» "<K 



0, ••,*• aO ^ *e> M 









**o« 













* o 




V 











\<? -\ 
















1 ^L'sLt* * 



°»° <$>' Y+ " y <F *U & 








S.'"^ 




V^^'a**' 























^ 











■? 



K o_ \yw* % * >> 








w 



*' . 













A TREATISE 



ON THE 



NEKVOUS DISEASES OF 

CHILDEEN 

FOR 

PHYSICIANS AND STUDENTS 

BY 

B. SACHS, M.D. 

ALIENIST AND NEUROLOGIST TO BELLEVUE HOSPITAL; NEUROLOGIST TO THE MT. SINAI HOSPITAL; 

CONSULTING PHYSICIAN TO MANHATTAN STATE HOSPITAL EAST, AND WEST; FORMERLY 

PROFESSOR OF MENTAL AND NERVOUS DISEASES IN THE NEW YORK. POLYCLINIC; 

EX-PRESIDENT OF THE AMERICAN NEUROLOGICAL ASSOCIATION 



SECOND EDITION, REVISED 



NEW YORK 

WILLIAM WOOD AND COMPANY" 

MDCCCCV 



pies rfecetweu 

■Joiwrifffti ciftrjf 

CK. Ms, tiOk 4 

&gfv a. 



J? 






Vfe 



Copyright, 1905, by 
WILLIAM WOOD AND COMPANY 



PREFACE TO SECOND EDITION. 



The author has reason to be pleased with the praise 
bestowed upon the first edition of his book in this country 
and in Europe. Translations into German and Italian have 
helped to extend its teachings, and a French edition is to 
appear shortly. Recent writers of text-books on Medicine 
and on Pediatrics have made liberal use of it. It is evident 
that the book has filled a distinct gap in medical literature. 

In preparing the second edition the writer has endeav- 
ored to reduce the size of the book without lessening its 
value as a special treatise on the nervous diseases of chil- 
dren. He has omitted the chapters on Anatomy and 
Physiology, the subject matter of which can readily be 
found in the text-books of Dana, Gowers, Starr and Oppen- 
heim. All detailed histories of cases and all bibliographies 
have been omitted. The names of authors have, however, 
been inserted in the text to give credit for work done, — 
a bit of justice which a writer should be happy and ready 
to do to others. 

Every chapter has been carefully revised and much new 
matter has been introduced. A comparison of the two 
editions of this book will show that important advances 
have been made in Neurology and the allied sciences with- 
in recent years. 

The author wishes to express his especial thanks to his 
publishers for their interest in this new edition, and to 
Dr. Alfred Wiener, of this City, for his assistance in pre- 
paring the index. 

21 East Sixty-fifth Street. 
June, 1Q05. 



CONTENTS. 



CHAPTER I. 

PAGE 

Introduction — Methods of Examination, i 

Examination schemes, 3; cranial measurements, 5; Preyer's observations, 
7; visual tests, 8; action of muscles, 11-34; gait, 35; sensory distribu- 
tion, 36; reflexes, 40; electrical examinations, 42; lumbar puncture, 46. 



PART I. 

GENERAL NERVOUS DISEASES. 

CHAPTER II. 

Convulsions — Eclampsia Infantum, 51 

Causes, 52; theories, 55; symptoms, 58; Laryngismus stridulus, 59; diag- 
nosis of convulsions, 62; treatment, 64. 

CHAPTER III. 
Epilepsy, • 67 

Symptoms of "grand mal," 68; "petit mal," 70; causes, 71; diagnosis, 
74; prognosis, 76; pathological anatomy, 77; treatment, 80; surgical 
treatment, 83. 

CHAPTER IV. 
Hysteria, 86 

Psychic or mental hysteria, 87; motor manifestations, 89; distinction be- 
tween epilepsy and hystero-epilepsy, 92; hysterical paralysis, 94; apho- 
nia, 95 ; sensory symptoms, 97 ; visceral hysteria, 100 ; diagnosis of 
hysteria, 102; pathology, 103; duration and courses, 103; treatment, 
104, 



VI COXTENTS. 



CHAPTER V. 
Chorea, 108 

Etiology, 1 08; causes, no; symptoms, 112; laryngeal chorea, 114; com- 
plications in chorea, 116; duration, 117; diagnosis, 118; morbid anat- 
omy, 119; prognosis, 123; treatment. 124. 



CHAPTER VI. 
Choreiform Diseases, ... 129 

Hereditary or Huntington's chorea, 129; symptoms, 130; pathological 
anatomy, 132; treatment, 133; Hereditary chorea without dementia, 
133; Habit chorea, 134; complex Co-ordinated movements (complex 
tics), 134; Gyrospasms of the head, 135; Chorea electrica, 136; Mala- 
die des tics convulsifs, 137; treatment, 138; Thomsen's disease, 139; 
Congenital Paramyotonia, 141; Paramyoclonus multiplex, 141. 

CHAPTER VII. 
Tetanus, . ... 143 

Etiology, 143; symptoms, 145; tetanus neonatorum, 147; pathological 
anatomy and morbid pathology of tetanus, 149; differential diagnosis, 
152; prognosis, treatment, 153. 

CHAPTER VIII. 
Tetany, 156 

Symptoms, 157; etiology, 159; symptoms of latent period, 160; differen- 
tial diagnosis, 161; morbid anatomy and pathology, 161; treatment, 
163; tetanoid chorea, 164. 

CHAPTER IX. 
Headaches, 166 

Classification, 166; anaemic headaches, 167; neurasthenic headaches, 169; 
headaches due to transitory hyperemia, 170; gastric headaches, 171; 
headaches due to genital irritation, 171; to ear disease, 171; to organic 
disease of the brain, 172; in acute infectious diseases, 173; malarial 
headaches, 173; uraemic headaches, 173; toxic headaches, 174; those 
due to eye-strain, 174; Migraine, 175; etiology, 178; pathology, 179; 
relation to eye-strain, 181; diagnosis, 182; prognosis, 182; treatment, 
183. 

CHAPTER X. 
The Disorders of Sleep, 186 

Normal sleep, 186; causes of insomnia, 187; Pavor nocturnus, 188; Enu- 
resis nocturna, 190; Somnambulism, 191. 



CONTENTS. Vll 



CHAPTER XI. 

PAGE 

Vasomotor and Tropho-neuroses, 192 

Exophthalmic goitre, 192; symptoms, 192; morbid anatomy, 196; treat- 
ment, 197; Thyroidectomy, 198; Thyroid enlargement at the age of 
puberty, 199; Tachycardia, 199; Myxcedema, 199; Angio-neurotic 
oedema, 201; Raynaud's disease, 203; Facial hemiatrophy, 204. 



PART II. 

ORGANIC DISEASES OF THE NERVOUS SYSTEM. 

CHAPTER XII. 

Diseases of the Peripheral Nerves, 209 

Some peripheral palsies, 209; brachial plexus lesions, 210; Erb's, 210; 
Obstetrical palsies, 210; pathology, 211; diagnosis, 212; prognosis, 
213; treatment, 214; Paralysis of the lower limbs, 215; Facial palsy, 
216; treatment, 223; other peripheral palsies, 225; Spasm, Tic convul- 
sif, Wry neck, 225. 

CHAPTER XIII. 
Multiple Neuritis, 227 

Symptoms, 227; course, 232; toxic forms, 233; toxasmic forms, 234; 
malarial neuritis, 234; pathological anatomy of multiple neuritis, 235; 
diagnosis, 236; treatment, 238; Diphtheritic paralysis, 240; pathologi- 
cal anatomy, 243; diagnosis, 244; treatment, 248; Lead paralysis, 246; 
diagnosis and treatment, 248. 

DISEASES OF THE SPINAL CORD. 

CHAPTER XIV. 

Infantile Spinal Paralysis — The Essential Paralysis of Chil- 
dren — Poliomyelitis Anterior Acuta, 249 

Symptoms, 240; distribution of paralysis, 252; deformities, 256; morbid 
anatomy and pathology, 258; theory of the disease, 263; differential 
diagnosis, 264; prognosis, 267; treatment, 268; Subacute anterior 
poliomyelitis, 270. 



Vlll CONTENTS. 



CHAPTER XV. 

PAGE 

Acute Myelitis, ... . . . . 272 

Symptoms, 272; origin of myelitis, 277; pathology and morbid anatomy, 
278; differential diagnosis, 282; prognosis, 284; treatment, 285; in- 
juries of the spinal cord, 288; pathology, 290; treatment, 291. 



CHAPTER XVI. 

Syphilis of the Spinal Cord: Specific Myelitis and Meningo-mye- 
litis, 293 

Symptoms, 293; Erb's type, 294; hereditary syphilitic affections, 296; 
differential diagnosis, 297; morbid anatomy, 298; prognosis, 301; 
treatment, 302. 

CHAPTER XVII. 
Disseminated Sclerosis, . . 304 

Symptoms, 304; pathological anatomy, 308; atypical forms and differen- 
tial diagnosis, 310; paralysis agitans and multiple sclerosis, 311; prog- 
nosis and treatment, 313. 



CHAPTER XVIII. 

Compression of the Spinal Cord — Pott's Paralysis, . . .315 

Causes of injury to the cord in Pott's disease, 315; symptoms of Pott's 
paralysis, 317; diagnosis, 318; prognosis, treatment, 319. 

CHAPTER XIX. 

Tumors of the Spinal Cord and its Meninges, .... 321 

Causes and symptoms, 321; pathology, diagnosis, 325; prognosis, treat- 
ment, 327; Syringomyelia and gliosis of the cord, 329. 

CHAPTER XX. 

Family Diseases of the Spinal or Cerebro-Spinal System, . . 333 

Hereditary ataxy. — Friedreich's disease, 333-334; symptoms, 334; differ- 
ential diagnosis, 339; pathological anatomy, 340; Hereditary ataxy 
(cerebellar type; type Nonne-Marie), 344; Hereditary spastic paraly- 
sis, 345; spinal type, 347; diagnosis, 348; cerebral type, 348; cerebral 
diplegia, 348; morbid anatomy of the cerebral type of hereditary spas- 
tic paralysis, 349; Little's disease, 350. 



CONTEXTS. ix 



CHAPTER XXI. 

PAGE 

Progressive Muscular Atrophies, 352 

Division of the subject, 353; distinction between progressive amyotro- 
phies and progressive myopathies, 354; type Aran-Duchenne, 355; He- 
reditary progressive muscular atrophy (Hoffmann), 357; Progressive 
neural muscular atrophy, etc., 359; etiology, diagnosis, 364; pathol- 
ogy, 366; Primary myopathies, 368; types of primary dystrophies, 
369; Muscula? pseudo-hypertrophy, 371; Erb's type, or the juvenile 
form, 374; Landouzy-Dejerine type, 374; diagnosis of dystrophies, 
377; physiological hypertrophy, 377; pathology, 379; histological 
changes in muscles, 380; duration, treatment, ^31 Total absence and 
early atrophy of muscles, 3S4. 

CHAPTER XXII. 

Malformations and Conditions due to Defective Development of 
the Cord, 386 

Amyelia, Atelomyelia, Asymmetry, Heterotopia, 386; Diplomyelia, 387; 
Spina bifida, 387; symptoms, 389; treatment, 390. 



DISEASES OF THE BRAIN. 

CHAPTER XXIII. 

Meningitis and Encephalitis, ... . . 391 

Simple acute meningitis, 391; etiology, morbid anatomy, 393; diagnosis, 
394; prognosis, treatment, 395; Tubercular meningitis, 397; onset, 
397; symptoms, 398; morbid anatomy, 400; pathology, diagnosis, 
401; prognosis, 402; treatment, 403; Epidemic cerebro-spinal menin- 
gitis, 404; microbic origin, 405; history of epidemics, 405; symptoms, 
407; morbid anatomy, etc., 409; differential diagnosis, treatment, 410; 
Meningitis due to traumatism after operation, 411; Meningitis due to 
ear disease, 412; Meningitis after infectious diseases, 414; after in- 
fluenza, 415; Septicemic meningitis, 415; Chronic basilar meningitis, 
415; Acute encephalitis, 416; Polio-encephalitis superior, 417; Bul- 
bar palsies, 418. 

CHAPTER XXIV. 
Hydrocephalus, 421 

Acute hydrocephalus, 421; meningitis serosa, 422; pathology of acute 
hydrocephalus, 422; chronic hydrocephalus, 423; congenital hydro- 
cephalus. 424; acquired internal hydrocephalus, 426; prognosis, treat- 
ment, 428; surgical methods, 429. 



CONTENTS. 



CHAPTER XXV. 



Diseases and Conditions due to Defective Development of the 
Brain, 

Larger defects, 501; Cyclops, 501; Anencephalus, Hemicephalus, 502; 
Porencephaly, 502; congenital porencephaly, 503; acquired poren- 
cephaly, 505; Microcephalus, 506; craniectomy, Lannelongue's pro- 
cedure, 509; Partial cerebral defects, 511; defective development of 
the occipital lobe, 511; Agenesis corticalis, 513; Macrocephalus, 514; 
Defective development of cranial nerve nuclei, 515; Pleuroplegia, 517. 



PAGE 



Infantile Cerebral Palsies (Spastic Hemiplegia, Diplegia, Para- 
plegia), 43° 

History of subject, 430; frequency, 431; distribution of paralysis, 433; 
onset, 434; etiology, 435; form of palsy, 438; rigidities and contrac- 
tures, 440; gait, 441; post-paralytic disturbances of motion, 441; atro- 
phy of muscles, 442; epilepsy with cerebral palsy, 444; idiocy with 
same, 444; classification of palsies, 447; morbid anatomy, 446; of 
congenital cases, 447; of birth palsies, 447; of acute cerebral palsies, 
449; polio-encephalitis, 454; differential diagnosis, 455; prognosis, 
456; treatment, 457; surgical treatment, 460; amaurotic family idiocy, 
462 ; symptoms, 463 ; diagnosis, 465 ; pathological anatomy, 465 ; prog- 
nosis, 468; treatment, 469. 



CHAPTER XXVI. 

Tumors of the Brain and its Meninges, 470 

Forms of tumor, locations of tumors, 470; etiology, 471; symptoms, 
472; Macewen's symptom, 474; tumors of cortex, 474; of frontal lobe, 
of third frontal convolution, of motor area, 475; of parietal lobe, of oc- 
cipital lobe, of temporo-sphenoidal lobe, 476; of basal ganglia, of crus 
cerebri, of the corpora quadrigemina, 477; of the pons, 478; of the 
cerebellum, 479; differential diagnosis, 480; pathology, 481; treatment, 
484; surgical procedures, 486. 

CHAPTER XXVII. 
Abscess of the Brain, ' . 488 

Occurrence of, 488; connection with ear disease, 489; with nasal disease, 
490; symptoms of abscess, 491; differential diagnosis, 493; prognosis, 
494; treatment, 495; surgical procedures, 495; Thrombosis of the in- 
tra-cranial sinuses, 497; symptoms, 498; special symptoms of throm- 
bosis, of cavernous, petrosal, and lateral sinuses, 499; treatment, 499. 

CHAPTER XXVIII. 



5oi 



CONTENTS. XI 



CHAPTER XXIX. 

PAGE 

Insanity, 519 

Differences between insanity of child and of adult, 519; frequency, 520; 
etiology, 520; forms of insanity, 522; Imperative concepts, 522; Mi- 
sophobia, 523; Delire du toucher, 523; Agoraphobia, Cerebral neu- 
rasthenia, 524; Hypochondriasis, 526; Mania, 527; Melancholia, 529; 
Periodic and circular insanity, 532; Dementia praecox, 533; Hebe- 
phrenia, 533; Katatonia, 533; Paranoia, 534; Moral insanity, 536; Ep- 
ileptic insanity, 537; Paretic dementia, 538; Masturbation and insan- 
ity, 539; prognosis, 540; treatment, 540. 

CHAPTER XXX. 
Idiocy and Imbecility, . 541 

Classification, 542; hereditary congenital idiocy, 542; developmental, 
acquired idiocy, 543; causes, 544; symptoms, 546; pathology, 551; 
prognosis, treatment, 553; Myxcedematous idiocy — sporadic cretinism, 
554; diagnosis, prognosis, treatment, 556. 

APPENDIX. 
Localization of the Functions of the Segments of the Spinal Cord, 561 

Index, 563 



THE NERVOUS DISEASES OF CHILDREN. 

CHAPTER I. 

INTRODUCTION— METHODS OF EXAMINATION. 

The nervous system of the child is subject to many dis- 
eases. Some of these are identical with the nervous dis- 
orders of tne adult ; others are peculiar to the early years 
of lite. 

The brain and the spinal cord do not attain their full 
development until months and years after birth, and even 
the peripheral nerves do not exhibit all their normal func- 
tions until the child is several weeks old. During this pe- 
riod of incomplete development, the nervous system re- 
sponds much more readily to morbid influences than it does 
in later years. This is especially true of the brain. It is in 
a state of irritability and instability, and a perversion of 
functions may result from causes Avhich would exert little 
or no influence over the nervous system of the youth or 
adult. Evidence of this is furnished by the behavior of a 
child in fever. The irregular choreiform twitchings and 
the delirium are often the outward signs of an unstable 
cerebral state, while the unusual irritability of the brain is 
proved by the occurrence of convulsions upon peripheral 
irritation to which the adult brain would not at all respond. 

In the early period of lite, too, hereditary affections of 
the nervous system are frequently manifest, and morbid 
psychic inheritance casts its shadows before. Inhibition of 
normal development may occur at any period ; family affec- 
tions are developed in the earlier years of life, and the 
acute infectious diseases of childhood are often followed by 
serious nervous disorders. If we add to these, diseases due 
to traumatism, we have an array of nervous disorders equal 



2 THE NERVOUS DISEASES OF CHILDREN. 

to, if not in excess of, those that occur after the age of 
puberty. Childhood is exempt only from the diseases due 
to senile deterioration, and is relatively free from those due 
to toxic agents, such as alcohol, metallic poisons, and syph- 
ilis ; but the effect of such diseases in the parent is exhib- 
ited with cruel persistence in the offspring. 

The diseases of the nervous system during the period of 
incomplete development are to be the special subject of 
this treatise. Before proceeding to the description of dis- 
ease it will be necessary to adopt a correct 

Method of Examination. — First of all inquire into the 
ancestry of the child. A reliable history of the physical 
and mental condition of parents, grandparents, and other 
relatives is of the greatest value in establishing a diagnosis. 
The habits and the diseases of the parents should be care- 
fully determined, for of the ills the child is heir to, not a few 
are due to alcoholism, to syphilis, to epilepsy, to hysteria, 
to psychic disorders of a parent. Next to heredity, en- 
vironment plays the most important part ; it is well, there- 
fore, to inquire into the home surroundings, the manner in 
which the child is watched and cared for; how it has been 
fed, trained, and educated. 

The previous history of the child is next in order. In 
every case inquire into the manner of its birth ; whether it 
was protracted or not ; whether or not instruments were 
used; whether the child was asphyxiated at birth or at 
once began to breathe freely. Make inquiries regarding 
the occurrence of spasms or convulsions ; the time at which 
the child began to take notice of things, to recognize parent 
or nurse, to stand, to walk, and to talk. The occurrence of 
other diseases, of the ordinary infectious diseases of child- 
hood, of whooping-cough, of pneumonia, of scarlet fever, 
of measles and meningitis, should be determined, and 
one should never forget to ask whether similar nervous 
conditions have been previously observed. Then proceed 
to the 

Examination of the Patient. — The art of making a 
diagnosis by mere inspection has gone out of date, and is 
cried down by many; yet I am willing to say that in fully 
one-half of the nervous diseases of children the nature of 



INTRODUCTION— METHODS OF EXAMINATION. 3 

the trouble can be suspected, if not made out, by a thor- 
ough inspection of the child without putting a finger to its 
body. I am not in favor of hurried examinations ; on the 
contrary, I wish to plead for the greatest accuracy in ex- 
amining for details ; but let the physician or student train 
his powers of observation and his diagnostic ability will be 
more acutely developed than that of the man who can never 
even suspect a disease unless he has all his tools (percus- 
sion-hammer, thermometer, assthesiometer, electrodes) con- 
stantly at his command. 

In my lectures to students I insist that they shall study the general ap- 
pearance of a child, and should not feel satisfied until they learn to recognize 
peculiarities of facial expression, of gait, and of stature ; to distinguish be- 
tween the behavior of the normal child and the feeble-minded, between 
spastic and flaccid palsies, and to determine by the peculiar deformity of the 
foot or by the scraping noise which the patient makes in walking, which 
group of muscles is affected. It is important from the history of the patient, 
and from these general observations to get correct first impressions ; these 
first impressions are then to be confirmed by a careful detailed examination. 

Never make a diagnosis unless the child has been wholly 
undressed ; if this is not done a Pott's paralysis may be 
taken to be a traumatic myelitis, or a neuritis may be mis- 
taken for poliomyelitis anterior. Lay the child on a table 
or on another person's lap in order to get a full view of it ; 
of the relative size of head and body ; of the proportionate 
development of arms, legs, and abdomen. Remember also 
that the child has heart and lungs, liver, spleen, and intes- 
tines, which, if diseased, may hold an important relation to 
the nervous disorder present. In proceeding to a detailed 
examination it is best to begin with the head, including the 
face, then take up the upper extremities, the abdomen, and 
finally the lower extremities. 

The following scheme includes the more important points to be estab- 
lished in the examination of a child ; the exact order of inquiry is subject to 
k slight modifications. 

EXAMINATION SCHEME. 

Head (Skull). — Size? Shape? Symmetrical? Dolichocephalic? Brachy- 
cephalic ? Fontanelles ? Hydrocephalus ? Bulging (Frontal or occipital) ? 
Position of head ? Stigmata of Degeneration (such as prognathism, de- 



4 THE NERVOUS DISEASES OF CHILDREN. 

formity of ears, hair-lip) ? Pain on percussion of skull ? Mental condition ? 
Speech? Sense of smell ? (use peppermint). 

Eyes (Fundus). — Vision ? Field of vision ? Pupils ? Light and ac- 
commodation reflexes ? Ocular movements ? Nystagmus (Lateral, vertical, or 
rotatory) ? Is the cornea sensitive ? Photophobia ? Hearing ? 

Face. — Symmetrical? Paralysis? Tongue? Taste? Deglutition? 
Articulation ? Sensation in face ? Teeth ? 

Upper Extremities. — Are they equally developed ? Position? Cir- 
cumference of arm and forearm? Movements (of shoulder, arm, forearm, 
wrist, fingers) ? Paralysis ? Tonus of muscles ? Are muscles atrophied or 
hypertrophied ? Reflexes ? Contractures ? Electrical reactions ? Sensation ? 

Spine. — Rigidity ? Lateral curvature ? Kyphosis ? Lordosis ? Pain on 
percussion ? 

Trunk. — Respiration ? Sensation ? Reflexes (Abdominal, Epigastric, 
Cremasteric) ? Action of muscles ? 

Lower Extremities. — Are they symmetrical ? Circumference of thighs 
and calves ? Ability to stand ? Romberg's symptom ? Ability to walk ? 
Gait (Paretic, Spastic-paretic, Ataxic, Cerebellar) ? Movements of individual 
groups of muscles? Is child able to raise thigh? To flex and extend 
thighs, legs, toes ? To stand on tip-toes ? To elevate toes, keeping heels on 
ground ? • Are muscles paretic or paralyzed, atrophied or hypertrophied ? 
Electrical reactions? Reflexes (Knee-jerks, Ankle clonus, Babinski reflex, 
plantar)? Contractures? Sensation? 

Vesical and Rectal Reflexes? 

Examination of the head of a child often gives us 
valuable information. The normal head should be well 
rounded and symmetrical. According to the age of the 
child the size will vary. The average horizontal circum- 
ference at birth (measured by a line passing from the gla- 
bella around the occipital protuberance) is between 38 and 
42 cm. ; at the end of one year between 45 and 52 cm., and 
in later years it may grow gradually to 56 cm. Any marked 
departure from these measurements is abnormal, but heads 
of tolerable size may be associated with deficient develop- 
ment of parts of the brain. I have seen cases with normal 
circumference in which the anterior defect was not evident 
in the measurement in consequence of a slightly excessive 
development of the occiput. A normal circumference is 
also present at times, although the actual cranial capacity 
may be very much diminished by a receding frontal bone. 

The following table will give the chief measurements of the skull in chil- 
dren ; some allowance should be made for the thickness of hair and scalp. 



INTRODUCTION— METHODS OF EXAMINATION 
Table of Cranial Measurements in Children. 



i. Circumfer- 
ence 



2. Binauricu- 
lar arc. . 



3. Volume ... 385 to 450 



New-born. 



M. 



34-o 



34.o 



End of 1st yr. 



M. 



42.0 



25-5 



4. Naso- occi- 

pital arc. 

5. Naso-breg- 

matic arc. 

6 Bregm atic 

lamb, arc. 



700 to 1,000 



20.0 22.0 



28.0 



7-7 



9.0 



7-7, 



9-a 



F. 



1st to 7th yr. 



M. F. 



42.034 to 46 ... 



25.0 



27 



10th year. 



M. F. 



49 



47 









1,300 


28.0 






10. 








10. 






12 



Taken around 
glabella and 
occipital pro- 
tuberance. 

Measured from 
B to opposite 
ext. aud. mea- 
tus. 

Volume is to cir- 
cumference as 
1,350 is to 50 
(in the adult). 



N, 0, T. 
12 |N, p. 
1/3 to A. 




Fig. 1. — Craniometrical Lines. (Benedict and Peterson.) 

The formula for the cephalic index is length : breadth :: 100 : x. An in- 
dex below 78 is dolichocephalic ; 78 to 80 mesocephalic ; above 80 brachyce- 
phalic. The facial length is determined by a line passing from N to lowest 
part of chin. 



Both halves of the head should be symmetrical. Asym- 
metry occurs chiefly in connection with defective develop- 



6 THE NERVOUS DISEASES OF CHILDREN. 

ment of the brain and with early cerebral lesions. (Fig. 
2.) The chief abnormalities of skull formation are as fol- 
lows: Dolichocephalus, a long skull, the anterior poste- 
^^--^^^ r i° r diameter being pro- 

>"^/'i\ ^^n. portionately greater than 

S^ / \ >v the transverse. Many new- 

/ / \ \ born children are dolicho- 

/ / \ \ cephalic as the result of 

/ / \ 1 compression of the head 

J / \ ] in the pelvic canal, but af- 

I / \ I ter a few days or weeks 

J/ \j the head is well rounded. 

Fig. 2.— Asymmetry of Skull in a Male, aged Brachycephalic — the skull 
Six Years. Right Hemiplegia from Birth. j s snort in the anterO-pOS- 

terior diameter. The terms 
microcephalus and macrocephalus need no further expla- 
nation. * 

Bulging of the frontal or occipital bones is important 
as an indication of hydrocephalus. If there is a very con- 
siderable increase of intracranial fluid the sutures may 
be pushed asunder and can be felt distinctly through the 
scalp. This same condition occurs in some cases of neo- 
plasm. In passing the hand over the head the fontanelles 
can be felt. The occipital fontanelle should be closed after 
a few weeks, the anterior remains open until the tenth or 
twelfth month. If it closes long before this period there is 
premature ossification of the sutures ; if it remains open 
much longer, it is a probable sign of rickets. 

After the examination of the head we may pass at once 
to the inquiry into the mental condition of the child. Ac- 
cording to its age we must ask whether it recognizes 
its mother or nurse ; whether it has learned to play, to 
understand what is said to it ; whether it begins to imi- 
tate sounds, to articulate, etc. ; in short, whether it shows a 
normal awakening of the mind. In children a little more 
advanced in years it is necessary to determine whether the 
child is able to keep up with others of its age ; whether it 

* A few special terms have been in use for oddly shaped heads : Keel-shaped skull, 
scaphocephalus ; triangular skull, trigonocephalus ; steeple-shaped skull, oxycephalus; 
obliquely deformed skull, plagiocephalus. 



INTRODUCTION— METHODS OF EXAMINATION. 7 

has been able to acquire the ordinary rudiments of knowl- 
edge. The physician should assure himself of these points 
by a personal examination, and should not depend upon the 
statements of parents and relatives. 

A few extracts from Preyer's observations on his own 
child will show what may be expected of a normal child at 
different stages of its development : 

During First Month. — Recognizes difference between light and 
dark objects (even on first day) ; follows with its eyes object moved slowly 
before it (as early as eleventh day) ; begins to hear about the fourth day ; 
recognizes sounds toward end of first month ; learns to distinguish between 
bitter and sweet ; recognizes disagreeable odors ; first tears on twenty-third 
day during a crying spell ; expresses displeasure by turning head away, by 
shutting its eyes, and, of course, by crying ; begins to smile. 

During Second Month. — Recognizes human voices and direction 
from which sound comes ; turns head toward low sounds ; is quieted by 
song ; smiles when music is heard ; recognizes its mother. 

During Third Month. — Moves arms, expressive of pleasure ; listens 
attentively ; is able to support head a little ; uses definite sounds in crying. 

During Fourth Month. — Associated eye movements perfect ; stares 
at new objects ; recognizes strange surroundings ; reaches after distant ob- 
jects ; first attempt to sit upright. 

During Fifth Month. — Recognizes strangers as such ; likes to take 
hold of everything ; stretches out its arms to be taken up ; holds head 
straight ; sits alone ; moves legs as if to walk ; forms syllables. 

During Sixth Month. — Distinguishes faces ; stares at strangers ; 
smiles, if smiled at ; smiles with relatives, not with strangers ; turns its head 
toward a person leaving the room ; begins to creep ; " crows." 

During Seventh Month. — Follows objects dropping out of its 
hands ; recognizes its image in mirror with evident pleasure ; points with 
finger at pictures ; purposive movements ; associates persons and names ; 
extends hand when asked ; articulates a number of different sounds in cry- 
ing and in " lolling to itself." 

During Eighth Month. — Sits upright when it is carried ; some chil- 
dren attempt to stand and to walk. 

During Ninth Month.— Begins to imitate tunes ; laughs heartily ; 
begins to beg for things. 

During Tenth Month. — Takes an intense interest in its food ; rec- 
ognizes parent after absence of several days ; he begins to walk alone ; an- 
swers questions by motions and indicates where certain things are. 

During Eleventh Month. — Stands quite alone; pushes chairs; 
makes first attempt to repeat sounds impressed upon its mind ; begins to 
articulate its own name ; understands language fairly well. 

During Twelfth Month. — Imitates laughter of others ; stretches its 



8 



THE NERVOUS DISEASES OF CHILDREN. 



arms out to enforce its demands ; improvement in walking and standing ; 
looks at others attentively while they eat. 

During Fourteenth, Fifteenth, and Sixteenth Months.— In- 
dependent speech is acquired, and repeats spoken words easily ; in seven- 
teenth month may speak short sentences, using verbs ; from this time on 
there is steady improvement in memory of words and use of language. 

At two years child may learn to repeat rhymes, to detect colors, etc. 

If the mental condition of the child has been satisfac- 
torily determined, the special senses should be examined. 
" Does the child see ? " is a question often answered affirma- 
tively by the mother, when a closer examination proves 
that the child is totally blind. Mothers are easily deceived 
in this, for the restless movements of the eyes in young 
children are supposed to be purposive and part of the vis- 
ual act. To test vision use a candle or a taper, and pass it 
in front of the eyes at some distance from the head, so as 
to avoid heat sensations, and note whether the child fol- 
lows the light. Do not be misled by accidental movements 
and avoid using rattles, for the child may turn its eyes in 



OD 




White 

Blue 

Red 



,.... ......Millie Green 

Fig. 3.— Normal Visual Fields for Colors. 

the direction from which the sound issues without seeing 
the object. Use also simple substances of different colors. 
A blind child will not notice an approaching linger until 
the eyelashes have been touched. 

It is a matter of still greater difficulty to test the field 



INTRODUCTION— METHODS OF EXAMINATION. 



9 



of vision. In very young children it may be altogether 
impossible, but after a child has reached the age of five 
months or thereabouts, it may be possible to make a rough 
test of the visual field by passing objects from above and 
below, as well as from the sides, into the visual field, and 
noticing when the child begins to turn its eyes toward this 





FlG. 5.— Field of Vision in a Case of Left-sided Hemianopsia. The Shading Repre- 
sents the Blind Part ; the Oval Outline is the Average Normal Field. (Gowers.) 

object. The ordinary test as applied in the adult cannot 
be employed for children until they have developed con- 
siderable intelligence. 

The condition of the visual field is often overlooked and the defects re- 
ferred to are much more frequent than they are generally supposed to be. 
An examination should be made in all cases of cerebral palsies, and in those 
in which cerebral tumor is suspected. 

In cases in which a very complex examination seems desirable, and these 
are relatively few, special tests should be made for form and color. The 
hemianopsic defects as they occur in tumors, and in cerebral palsies (first 
described by Freud), the concentric limitations occurring in hysteria, are 
the most common defects in children. 

The pupils should be equal and contract promptly to light and during ac- 
commodation. They should also contract if the patient attempts to close 
the eyes while the examiner endeavors to keep the lids open (A. Westphal 
and Piltz). 

The ocular palsies will be best understood by reference to 
the subjoined table. Conjugate deviation is due to disease 
in the hemisphere, and according to Grasset to lesions in 
the first and second frontal convolutions and in the angular 
gyrus and its vicinity ; the patient looks toward the lesion 
except in the case of spasm of the muscles, when the patient 
looks away from the lesion. Nystagmus occurs in many 
cerebral affections, particularly in cases of early cerebral 
disease or congenital defect. It is seen also in cases in 



IO 



THE NERVOUS DISEASES OF CHILDREN. 



which there are stigmata of degeneration as well as in 
multiple sclerosis. In the latter it may be bilateral or 
rotatory. The corneal reflexes (the prompt closure of the 
eyelids if the cornea is touched carefully with the head of 
a pin or a small lead-pencil) should be tested. In a great 
many individuals this reflex does not ensue upon irritation 
of the conjunctiva, a fact that has deceived many physi- 



cians. 



Muscles of the Eyes and Face. 













Diseases in 


Name of 


Normal 


Symptoms of Defi- 


Innervated 


Represented 


which Muscle 


Muscle. 


Function. 


cient Action. 


by 


in 


is commonly 
Involved. 


Ciliary . . . 


Makes 1 e n s e 


Loss of accommo- 


The third 


Corpora 


1 c ^-S^: C 




more convex ; 


dation ; spasm off nerve. 


quadrigem- 


3 .52 fl w w 




aids in accom- 


ciliary muscle may 


ina and pe- 




modation. 


cause myopia. 


duncle. 


^§go 


Sphincter 


Contraction of 


Paralytic mydria- The third 


Corpora 


%:-%& a 


iridis. 


pupil to light 


sis ; no contrac- 


nerve. 


quadrigem- 


traumatism t 
theritic palsj 
n; tumor ; pc 
rior ; in cere 
itary ataxy ; 




and during 


tion to light and 




ina and pe- 




accommoda- 
tion. 


during conver- 
gence or accom- 




duncle. 






modation. 






Dilatato r 


Dilates pupils; 


Pupil does not re- 


Sympathetic 




pupillae, 


as a result of 


spond to sensory 






— 43 3 <U -rj 

.52 a£ o- « w 




sensory o r 
psychic stim- 


stimulation. 






*Z \=, 42 3 i- w> 




ulation. 








Meni 
eye 
ofm 
aliti: 
lis; 
by c 


Rectus su- 


Moves eye up- 


Upward movement 


The third 


Corpora 




perior. 


ward and in- 


limited; diplopia; 


nerve. 


quadrigem- 




ward. Acts false image 




ina and pe- 


.S TO 




with inferior 


above ; deficient 




duncle. 


0) 3 




oblique. 


rotation of eye- 
ball. 






.52 o. 


Rectus in- 


Moves eye in- 


Strabismus diver- 


As above. 


Corpora 




ternus. 


ward. 


gens ; defective 




quadrigem- 


TO </3 






inward pull. 




ina and pe- 
duncle. 


e « SI 


Rectus in- 


Moves eyelmperfect move- 


As above. 


Corpora 


£ fi3 


ferior. 


downward ment downward; 




quadngem- 


a « si 




and rotates it eye rotated out- 




ina and pe- 


• -"3 to 




inward. Acts 


ward. 




duncle. 




with superior 








u 5 <u 

O Sri 




oblique. 










Obliquus 


Acts with rec- 


Imperfect m o v e - As above. 


Corpora 


* 3 t/J * 


inferior. 


tus superior, 


ment upward; eye 




quadrigem- 


£3 




moving eye 


rotated inward. 




ina and pe- 




upward and 






duncle. 


•r-L >> <u 




outward, and 












rotates it up- 








— 1 TO O 

H J4 (J 




ward. 










Obliquus 


Moves eye On looking down- 


The fourth 


Peduncle 


•? 1!? 


superior. 


downward 


ward eye is pulled 


nerve (tro- 


near the 


T3 ° "E 




and outward. 


inward ; conver- 


chlearis). 


corpora 


£ -43 

, LO -< — » 




Acts with in- 


gent strabismus ; 




quadrigem- 


£•-£-£ 




ferior rectus ; 


diplopia on step- 




ina. 


8 3>£ 




rotates down- 


ping downward. 






Diseas 
menir 
after < 




ward. 



















INTRODUCTION— METHODS OF EXAMINATION 
Muscles of the Eyes and Face. — Continued. 













Diseases in 


Name of 


Normal 


Symptoms of Defi- 


Innervated 


Represented 


which Muscle 


Muscle. 


Function. 


cient Action. 


by 


in 


is commonly 
Involved. 


Rectus ex- 


Moves eye out- 


Outward move- 


The sixth 


Pons 


As before, and 


ternus. 


ward. 


ment impaired ; 


nerve (ab- 




in disease of 






head turned in di- 


ducens). 




the pons. 






rection of para- 












lyzed muscle. 








Levator 


Raises upper 


Ptosis ; eye closed; 


The thi r d Pedunc le 


Associated 


palpebrae 


eyelid. 


may be opened a 


nerve. 


and cor- 


with other 


superioris 




little by frontalis 




pora quad- 


third nerve 






muscle. 




rigemina. 


diseases; often 
congenital. 


Orbicular- 


Closes eyelids. 


Eyes cannot be 


The seventh 


Pons 


Peripheral fa- 


is palpe- 




closed (lagoph- 


nerve. 




cial neuritis ; 


brarum. 




thalmos). 






affections at 
base of brain 
(meningi t i s , 
tumor, and 
the like) ; in 
some dystro- 
phies. 


Fronta 1 i s 


Raises eye- 


Imperfect raising 


As above. 


As above. 


As above. 


and cor- 


brows ; fold- 


of eyebrows ; no 








ruga tor 


ing of skin of 


frowning ; lines of 








supercilii 


forehead, as 
in frowning. 


forehead d i s a p- 
pear. 








Orbicula- 


Move lips and 


Face distorted and 


The seventh 


Pons 


Cerebral apo- 


ris oris, 


cheeks, as in 


pulled toward 


nerve. 




plexies (es- 


buccin- 


speaking, kiss- 


healthy side ; ina- 






cape of upper 


ator, and 


ing, showing 


bility to pout lips 






branches of 


other 


of teeth, and 


or to whistle; drib- 






fa c i a 1 ) ; pe- 


muscl es 


the like. 


bling of saliva 






ripheral facial 


of face. 




from paralyzed 
side ; flapping of 
cheek with each 
expiration, owing 
to paralysis of 
buccinator ; d i s- 
app earance of 
nasolabial fold. 






palsy (of t e n 
due to expos- 
u re ) ; in all 
lesions of 
pons ; in dys- 
trophies. 


Masseter , 


Masseter, tem- 


Mastication imper- 


The fifth 


Pons 


Rarely affect- 


tempor- 


poral, and in- 


fect ; if in spasm, 


nerve. 




ed in children 


al, and 


ternal ptery- 


jaws cannot be 






except in tet- 


ptery- 


goid elevate 


opened; jaw 






anus ; in facial 


goids. 


lower jaw ; 


moved toward 






hemiatrophy; 




external pter- 


paralyzed side by 






in lesions 




ygoid draws 


action of ptery- 






of pons. 




jaw forward ; 


goids. 










the two ptery- 












goid muscles 












of one side 












acting togeth- 












er move teeth 












toward oppo- 












site side. 











Mydriasis can be caused by paralysis of sphincter iridis (third nerve), or 
by spasm of the dilator muscles (mydriasis spastica). Atropin, Duboisin, 
and Hyoscyamia have a similar effect by paralyzing the sphincter, and con- 



12 THE NERVOUS DISEASES OF CHILDREN. 

trading the dilator. Cocaine produces a transitory mydriasis by irritation of 
the pupillary fibres of the sympathetic. 

Myosis may be due to irritation of the sphincter supplied by the third 
nerve, and occurs in early stages of many cerebral affections, including 
apoplexy ; or it may be due to paralysis of the dilator muscle. 

Inequality of pupils is sometimes congenital, more often due, however, to 
cerebro-spinal disease (meningitis, syphilis, multiple sclerosis, hereditary ataxia). 
Muscles of both eyes act conjointly. If such action is deficient we speak of 
conjugated paralysis. There may be paralysis of lateral, upward or downward 
movements, without total palsy of any one muscle. If individual muscles 
are paralyzed, or muscles in one eye only, double vision is certain to result. 

In diplopia the exact position of true and false images will help to de- 
termine the character of the ocular palsy. If one muscle is paralyzed second- 
ary deviation of the associated muscle in the other eye may take place. 

Paralysis of the inner muscles of the eye is designated as ophthalmo- 
plegia interna ; paralysis of the muscles moving the eyeball, as ophthalmo- 
plegia externa, and such ophthalmoplegia may be total or partial. Total 
ophthalmoplegia externa and interna is the occasional accompaniment of dis- 
ease at the base ; partial ophthalmoplegia (only part of the muscles sup- 
plied by the third nerve, for instance, being affected) points to nuclear disease, 
but syphilitic infiltration of the root fibres may simulate nuclear disease. 

Narrowing of the orbital fissure (sometimes congenital) may be due to 
paralysis of smooth fibres in the lid, innervated by the sympathetic. Clonic 
movements of eye muscles we term nystagmus ; this may be lateral, vertical, 
or rotatory. 

An examination of the sense of hearing is often called 
for. In children of defective development this sense may 
be entirely wanting. The simplest tests are to clap the 
hands at a distance from the child's head, to whisper its 
name, to use a loudly ticking watch — even very young 
children will be attracted by the sound if it is perceived. 
In the case of older children tuning-forks maybe applied to 
the head. In disease of the ear proper the vibrations would 
be perceived, but not so if the nerve itself, the labyrinth, or 
the auditory tract were involved. Hyperacusis occurs in 
amaurotic family idiocy, in some forms of meningitis. 

Asymmetry of the face may point to congenital defect 
or to a preceding palsy. It is of some interest to know 
that asymmetry of the face and of the nose, adhesion of the 
ear-lap, malformations of the outer ear, the existence of 
a torus palatinus (felt as a ridge in the roof of the mouth) are 
the ordinary stigmata of a degenerative type, of which so 
much has been made by the Italian criminologist of the 



INTRODUCTION— METHODS OF EXAMINATION. 



13 



present day. If such stigmata prove a predisposition to 
the development of nervous troubles, to crime, or to in- 
sanity, it is well that the physician should know of them. 

The teeth play a very important part in the life of every child. If not de- 
veloped between the ages of six months and one year, rickets must be sus- 
pected. Notched teeth (second dentition) are suspicious of syphilis. Unusually 
early development of the teeth is an evidence of premature ossification. 

. Muscles of Tongue, Palate, and Pharynx. 













Diseases in 


Name of 


Normal 


Symptoms of Defi- 


Innervated 


Represented 


which Muscle is 


Muscle. 


Function. 


cient Action. 


by 


in 


commonly In- 
volved. 


Genio- 


Pushes tongue 


Tongue when pro- 


The twelfth 


Medulla 


• 




glossus. 


to opposite truded deviates to 
side. paralyzed side. 


nerve (hy- 
poglossal). 








Styloglos- 


Raises tongue Tongue cannot be 


The twelfth 


Medulla.... 


Bulbar pal- 




sus. 


backward 
and upward. 


moved backward 
or hollowed out 
(action deficient 
in many healthy 
subjects). 


nerve. 




sies (acute 
and chron- 
ic) ; in spe- 
cific and tu- 
fa ere ular 




Lingual 


All movements 


When lying in 


The twelfth 


Medulla.... 


diseases of 


• 


muscle 


of the tongue 


mouth deviation 


nerve. 




base ; dys- 




proper. 


itself. 


to healthy side ; 
when protruded 
deviates to paral- 
yzed side ; if one 
or both halves are 
atrophied tongue 
looks shrivelled. 






rophies 
(rare). 




A z y g s 


Shortening of Uvula deviates. to- 


P r b a b lylMedulla .... 


As above 


uvulae. 


uvula. 


ward sound side ; 
if both sides are 
paralyzed there is 
nasal tone and 
regu r g i t a t i n 
through nose. 


pharyngeal 
plexus; 
seventh 
nerve (?). 






L e vat r 


Raises the vel- 


Arch cannot be 


As above. 


Medulla.... 


As above; see 


palati. 


um palati. 


raised in the in- 
tonation of "ah; " 
if paralysis is bi- 
lateral flapping of 
arch and regurgi- 
tation of food 






also seventh 
nerve affec- 
tions. 






through nose. 






Palato- 


Prevent food 


Regurgitation of 


The fifthPons 


Basilar affec- 


pharyn- 


from passing 


food; nasal 


nerve. 




tions. 


geal mus- 


toward up- 


speech. 








cles. 


per part of 
pharynx and 
posterior 
nares. 










Stylo-pha- 


Helps to draw 


Imperfect degluti- 


Glosso -pha- 


Medulla.... 


Bulbar affec- 


ryngeus. 


larynx up- 


tion ; food gets 


ryngeal. 




tions and dis- 




ward so as to 


into windpipe. 






eases of the 




be closed by 








base. 




epiglottis and 












ov ertopp e d 












by tongue. 













14 



THE NERVOUS DISEASES OF CHILDREN. 

Muscles of Tongue, Palate, and Pharynx. — Continued. 













Diseases in 


Name of 


Normal 


Symptoms of Defi- 


Innervated 


Represented 


which Muscle is 


Muscle. 


Function. 


cient Action. 


by 


in 


commonly In- 
volved. 


Constric- 


Help to push 


Food is swallowed 


P h a ryngeal 


Medulla. . . . 


Diseases of the 


t o r s of 


food into gul- 


very imperfectly ; 


plexus. 




base (bulbar). 


pharynx. 


let. 


sticks in throat. 








Laryngeal 


Movements of 


Hoarseness and 


Recurrent 


Medulla .... 


Bulbar troub- 


muscles. 


vocal cords in 


difficulty in 


lar yngeal 




les (similar 




respirat ion 


breathing ; laryn- 


nerve ex- 




symptoms 




and in articu- 


goscopy examina- 


cepting the 




maybe caused 




lation. 


tion reveals false 


eric o- thy- 




by tumors and 






position of vocal 


roid mus- 




foreign bodies 






cords (see special 


cle. 




in larynx). 






text-books). 









The movements of the tongue have been considered in the annexed table. 
A short frenulum may cause difficulty in articulation, but it is often suspected 
to be the cause in cases in which the defect is due to a distinct cerebral 
lesion. 

Sensory disturbances of the face are rare in children, but, if suspected, 
tests should be made carefully with cotton, with the head and point of a pin, 

and by application of hot and cold ob- 
^ jects. Subjective sensory disturbances 

^T'ir^t^ ^& tti (neuralgia) may occur as in adults, and 

will vary in distribution according to 
the branches affected. (Fig. 6.) 

In examining the trunk and 
the four extremities no fact is 
more important to establish 
than the existence of paresis, 
or paralysis of individual mus- 
cles, or of groups of muscles. 
To be able to do this the ex- 
aminer must know the action 
of all the more important mus- 
cles in health and the disturb- 
ances due to disease of such 
muscles. These are the very 
facts in which the student's knowledge is, as a rule, most 
defective. In the appended tables I shall endeavor to 
give the principal points in concise fashion : 




Fig. 6.— Sensory Chart of Face. 
(C. S. Freund.) 



INTRODUCTION— METHODS OF EXAMINATION 



15 



Muscles of Head and Neck. 



Name of 
Muscle. 



Normal 
Function. 



mastoid. 



Sterno-Raises and 
c 1 e i d o- turns face to 
opposite side; 
head inclines 
to same side ; 
if both mus 
cles act con 
jointly head is 
brought for- 
ward. 



R e c t u s To flex head, 
c api tis 



Symptoms of Defi- 
cient Action. 



anticus 
major. 



Rectus 
capitis 
anticus 
minor. 



To flex head. 



Rectus Slight rota 
capi ti s; tion. 
lateralis. 



Scalenil 
anterior 
medi- 
u s , e t 
po s t e- 
rior. 



L o ngus 
colli. 



Elevate ribs 
when verte- 
bral column 
is fixed ; aid 
in inspira- 
tion ; slight 
lateral flex- 
ion. 

Flexor of ver- 
t e b r a 1 col- 
umn. 



Inability to raise 
head from bed, or 
other horizontal 
position, if both 
muscles are affect 
ed ; if one muscle 
is affected, no 
marked change of 
position, unless 
opposite muscle 
is contractured 
spasm of muscle 
frequent ; head in- 
clined to one side 

Cannot flex head 1 
so as to bring 
chin on chest. 



Innervated 
by 



Deficient rota- 
tion scarcely 
noticeable, un- 
less sterno-clei- 
do-mastoids are 
diseased. 

Deficient inspi- 
ratory move- 
ments. 



Imperfect flexion 
of upper spine, j 



Spinal ac 
cessory. 



Represented 
in 



Upper cervi 
cal. 



Lower cervi- 
cal nerves. 



Lower cervi- 
cal nerves. 



Medulla and 
second and 
third cer- 
vical seg- 
ments. 



Diseases in 
which Muscle is 
commonly In- 
volved. 



Upper 


cer- 


vical 


seg- 


ments. 




Lower cervi- 


c a 1 seg- 


ments. 





In bulbar and 
cervical cord 
affections ; in 
later stages of 
progressive 
muscular 
atroph ies ; 
o c c asionally 
in neuritis. 



Diseases of 
the cervical 
region (my- 
elitis, raen- 
i n g i t is, 
tumor ; pro- 
gre s s i ve 
wasting of 
muscles. 



The sterno-cleido-mastoid and the other muscles of the head and neck, 
mentioned in these two tables, are more frequently the seat of spasm than of 
paralysis. The spasm may be restricted to one muscle or may involve 
several. It may be the result of organic disease in the medulla oblongata 
or spinal cord. More often it is functional in character and related to other 
spasmodic conditions. The same group of muscles may be involved in the 
ordinary rheumatic affections (torticollis, caput obstipum). 



i6 



THE NERVOUS DISEASES OF CHILDREN. 
Muscles of Shoulders and Upper Extremity. 



Name of 
Muscle. 



Trapezius. 
i. Cla - 
v i c u 1 ar 
portion 
(respi- 
ratory; 
outer 
third ot 
c lavicle 
to occipi- 
tal bone). 



Normal 
Function. 



Pulls head 
backward; ro- 
tates slightly 
toward side ot 
muscle , so 
that chin is 
turned to op- 
posite side 
contraction ot 
both clavicu- 
lar portions 
bends head 
back ward 
slight eleva- 
tion of shoul- 
ders ; aids in 
deep inspira- 
tion. 

Raises shoul- 
der - blade ; 
elevation of 
acromion 
(clavicle goes 
along). 



Symptoms of Defi- 
cient Action. 



2. Middle 
por tionj 
(fro ml 
acromion! 
and outer 
spine o f 
scapula to 
ligament. 
nuchae 
and up- 
per dorsal 
spines). 



3. Low e r Adduction ot 
portion scapula to- 
and ad- ward median 
ductor. line. 



R h o m 
boids. 



Oblique move 
ment of scap 
ula from be- 
low, upward 
and inward, 
so that infe- 
rior angle is 
brought near- 
er the medi- 
an line ; hold 
spinal margin 
of scapula 
down to tho- 
rax. 



Deficient back- 
ward movement 
of head; not 
marked as rule 
because deep 
muscles perform 
this function 
shoulder does not 
move during in- 
spiration. 



Acromion depress- 
ed by weight oi 
upper extremity ; 
inner upper an- 
gle may be pulled 
upward by levator 
anguli scapulas ; 
internal lower an- 
gle is nearer to 
median line. 



Margin of scapula 
is about ten ctm 
distant, instead of 
being five or six 
ctm. distant from 
median line ; loss 
of adductor may 
be covered up by 
action of rhom- 
boids ; rounding 
of back. (Fig. 8.) 

Deep groove be- 
tween inner mar- 
gin of scapula and 
thorax ; if serra- 
tus is normal, this 
groove disap- 
pears if arm is ex- 
tended forward ; 
shoulder -bl a d e 
cannot be approx- 
imated to median 
line. (According 
to Duchenne this 
can be affected by 
upper portion of 
latissimus dorsi.) 



Innervated 
by 



Spinal ac 
cessory. 



Spinal ac 
cessory 



Spinal ac 
cessory 
nerve. 



Fifth cervi 
cal. 



Represented 



Medulla 
and second 
and third 
cervical 

segments, 



Diseases in 
which Muscle is 
commonly In- 
volved. 



As above. 



Medulla 
and second 
and third 
c e r vi c al 
segments, 



Fourth and 
Fifth cervi- 
c al s e g 
ments. 



Progressive 
muscular 
wasting; dis- 
eases of me- 
dulla and up- 
per cervical 
cord; clavi- 
cular portion 
least fre- 
quently in- 
volved. 



As above. 



As above. 



INTRODUCTION— METHODS OF EXAMINATION, 



17 



Frontalis 

Facial Nerve 

Corrugator Supercilii 

Orbicular. 

Palpebr. 

Muscles of Nose 

Zygomatici 

Orbicularis Oris 

Median Division of I 

Facial Nerve f 

Masseter 

Levator Menti 



Hypoglossal Nerve 

Lower Facial Nerve 

Platysma Myoides 

Hyoid Muscles' 



Omohyoid 



Anterior Thoracic Sup- ) 
plying Pectoral Mus- v 
cles ) 




( Region of Central 
( Convolutions 



Speech Area 
Temporal 

Facial 

Trunk of Facial Nerve 

( Posterior Auricular 

I Nerve 

Facial 

Lower Branch 
Splenius 

Sternocleido Mastoid 
Spinal Accessory Nerve 
Levator Anguli Scapulae 

Trapezius 
Scapular Branch 

Circumflex Nerve 
Posterior ThoracicNerve 



Phrenic Nerve Erb's Point Brachial Plexus 

Fig. 7. — Motor Points of Face. (Erb.) 




FlG. 8. — Patient with Hypertrophy of Infra and Supra Spinati, Showing Rotation of 
Right Scapula and Deep Groove along Inner Margin of Scapula (Atrophy of 
Rhomboids and Slight Atrophy of Lower Portion of Trapezius). 



1 8 THE NERVOUS DISEASES OF CHILDREN. 

Muscles of Shoulders and Upper Extremity. — Continued. 



Name of 
Muscle. 



Levator 
an g ul i 
scapulae. 



Serr a t u s 
magnus. 



Deltoid 

(three di- 
visions). 



Normal 
Function. 



Infraspi- 
natus. 
Teres 



Sub scap- 
ularis. 



S u praspi- 
natus. 



Draws superi- 
or inner angle 
of scapula up- 
ward : aids 
in shrugging 
of shoulders. 

Rotation o i 
shoulder- 
blade out- 
ward , and 
slight eleva- 
tion of acro- 
mion ; holds 
inner margin 
of scapula to 
thorax; brings 
arm from hor- 
izontal to ver- 
tical position. 



To raise arm 
to horizontal 
position, and 
forward, out- 
ward, or back- 
ward ; move- 
ments possi- 
ble only if 
scapula is 
fixed by ac- 
tion of serra- 
tus and tra- 
pezius. 

Rotator hu- 
meri posticus 
(Duchenne) ; 
rotate arm: 
outward. 

Rotator hume- 
ri an ticus! 
(Duchenne) ; I 
rotates arm 
inward. 

Helps to' 
steady shoul- 
der-joint and 
to elevate 
arm forward 
and outward ; 
outer angle of 
scapula is de- 
pressed. 



Symptoms of Defi- 
cient Action. 



Isolated paralysis 
rare. 



Scapula pulled up- 
ward ; lower inner 
angle nearer the 
median line ; arm 
cannot be raised 
above horizontal 
position ; if arm is 
stretched forward 
scapula is re- 
moved from tho- 
rax ("winged 
scapula ") ; dur- 
ing abduction of 
arm, scapula is 
moved nearer to 
median line, and 
crowds trapezius 
and rhomboids 
forward. 

Can raise shoulder 
but not arm; 
shoulder flattened 
(atrophy) ; groove 
between acromion 
and head of hu- 
merus • each di- 
vision of deltoid 
may be paralyzed 
singly. 



Arm cannot be 
moved outward. 

Difficulty in writ- 
ing (Duchenne). 

Arm cannot be 
moved inward ; 
scapula is rubbed 
against ribs. 



Innervated 
by 



Third and 
Fifth cervi- 
cal nerves. 



Posterior 
thoracic 
nerve. 



Represented 



Second and 
Fourth (?) 
c er vi cal 
segments. 



F i fth and 
sixth cervi- 
c al seg- 
ments. 



Diseases in 
which Muscle is 
commonly In- 
volved. 



Circumflex. 



Supra - 
scapular. 

C ire um- 
flex. 

Subscapu- j 
lar nerve. I 

J 



According to Du-Suprascap 



chenne, humerus 
is separated still 
further from acro- 
mion, if supra- 
spinatus is affect- 
ed in addition to 
deltoid. 



ular. 



Fourth, fifth, 
and sixth 
cervical 
segments 



Fourth, fifth, 
and sixth 
cervical 
segments. 



Fourth 
vical. 



D y s t r o phies 
and cervical 
diseases. 



Progressive 
muscular at- 
rophies (dys- 
trophies); 
ne u r i t i s of 
part of the 
brachial plex- 
us; after trau- 
matic injuries 
to shoulder ; 
i n cervical 
cord affec- 
tions. 



As above; also 
in Erb's form 
of obstetrical 
paralysis. 



As in case of 

deltoid. 



As above. 



INTRODUCTION— METHODS OF EXAMINATION 



19 





20 THE NERVOUS DISEASES OF CHILDREN. 

Muscles of Shoulders and Upper Extremity.— Continued. 



Name of 
Muscle. 



La t issi- 
raus dor- 
si. 



Teres ma- 
jor. 



Pectoralis 
major. 



Normal 
Function. 



Pulls the arm, 
when raised, 
downward 
and back- 
ward ; if arm 
is at rest up- 
p e r portion, 
brings scap- 
u 1 a nearer 
the median 
line ; united 
action of up- 
per third of 
both muscles 
causes exten- 
sion of dorsal 
trunk ; single 
action causes 
lateral move- 
ment of trunk. 

Rotates raised 
humerus in- 
ward; adduc- 
tion of arm *o 
thorax ; sligh 
elevation of 
shoulder. 

Clavicular 
portion d e 
presses hu 
merus 
raised 
tion to 
zontal ; 
d u c t i o n of 
arm, as in 
giving a bless 
ing ; sternal 
p o r t i on de- 
presses arm 
c o m p letely, 
and if arm 
at rest draws 
acromion for- 
w a r d and 
backward 



from 
posi- 

hori 
ad 









Diseases in 


Symptoms of Defi- 


Innervated 


Represented 


which Muscle is 


cient Action. 


by 


in 


commonly In- 
volved. 


Arm cannot be 


Sub scapu- 


S ixth and 


As in progres- 


moved backward ; 


lar, also; seventh cer- 


sive atrophies 


insufficient exten- 


branches of 


vical. 


and dystro- 


sion of dorsal 


dorsal and 




phies ; in cer- 


spine ; trunk can- 


lumbar 




vico-dorsal 


not be moved lat- 


nerves pass- 




lesions; in 


erally. 


ing through 
muscle. 




neuritis. 


Very few symp- 


S u b s c apu- 


Seventh cer- 


As above. 


toms ; action sup- 


lar. 


vical. 




plied by other 








muscles. 








Imperfect adduc- 


Anterior 


Fifth, sixth, 


Amyotrophies 


tion of arm ; par- 


thoracic. 


and seventh 


and dystro- 


alysis can be dis- 




cervical. 


phies, chiefly; 


covered best by 






also in lesions 


extending arms 






of brachial 


and tryingto press 






plexus. 


volar surfaces 








agai n s t each 






' 


other. 









Absolute paralysis of both upper extremities is rare in children. If an 
entire extremity is paralyzed, the arm either hangs limp by the side of the 
body, or is flexed at the elbow. If the arm is lifted it falls by its own weight. 
Children sometimes refuse to make an effort, or do not understand what is 
wanted of them. After a few trials it is generally possible to decide whether 
there is a loss of power or mere lack of effort. Total paralysis of the upper 
extremity occurs either from disease of the brachial plexus, from spinal cord 
affections, or from cerebral lesions. If due to the first or second causes, the 



INTRODUCTION— METHODS OF EXAMINATION. 21 




FlG. II. — Young Boy with Multiple Neuritis, showing Double "Wrist Drop" and 

Slight " Foot Drop." 




FlG. 12. — Appearance of Hand in an Early Stage of Progressive Muscular Atrophy, 
Atrophy of Abductor Brevis and Opponens Pollicis. (Duchenne. ) 



22 



THE NERVOUS DISEASES OF CHILDREN. 



paralysis is flaccid ; if due to the third cause, the paralysis is spastic, and 
very often part of a hemiplegia. Plexus lesions are observed in very young 
children (obstetrical palsies), spinal lesions in children of any age, and cere- 
bral lesions in children under the age of four chiefly ; but the after-effects of 
these conditions are often visible late in life. From the causes enumerated 
above, as well as in cases of neuritis and progressive muscular diseases, the 
paralysis maybe incomplete and restricted to definite groups of muscles only. 
To test the extent of paralysis, the patient should be asked to perform the 
various movements, such as raising the shoulder, putting out arm, flexing 
and extending the elbow, wrist, and fingers. In very young children these 
tests cannot always be satisfactorily made, but even in infants much can be 
inferred from their power to hold or to grasp objects placed in front, above, 
or below them. In older children further tests can be made by offering re- 
sistance to active movements, and asking them to overcome it. The ability 
to bring the fingers in opposition to one another, to make the thumb touch 
the tips of the other fingers, the moving of the fingers to and from the middle 
one, and the ability to write, are necessary tests of the action of the intrin- 
sic muscles of the hand. 

Muscles of Arm, Forearm, and Hand. 













Diseases in 


Name of 


Normal 


Symptoms of Defi- 


Innervated 


Represented which Muscle is 


Muscle. 


Function. 


cient Action. 


by 


in commonly In- 
volved. 


Triceps. . . 


Extends fore- 


Arm cannot be ex- M usculo- 


Six th, s e v- 


• Poliomyelitis 




arm ; 1 o n g 


tended except by spiral. 


enth, eighth 


and other 




head of t r i- 


its own weight ; if 


cervi c a 1 


affections of 




ceps, and cor- 


long head of tri- 


segments. 


cervical 




aco-brachialis 


ceps is affec ted 






cord ; trau- 




help to keep 


subluxa t i o n of 






matic inju- 




head of hu- 


head oi humerus 






I ries ; amyo- 




merus in posi- 


occurs easily. 






\ trophies 




tion. 








j and dystro- 


Biceps 


Flexion and 


Flexion deficient, Musculo-cu- 


Fourth, fifth, 


| phies (t r i- 




supination of 


but can be carried taneous. 


sixth cervi- 


ceps es- 




forearm. 


out in part by 
other muscles. 




cal. 


capes in 

many pe- 

1 r i ph er al 










j palsies). 


Supinato r 


Flexes fore- 


Flexion and prona- Musculo- 


Fourth, fifth As above ; in- 


longus 


arm and aids 


tion de fi c i e n t ; spiral. 


cervical, j volved in pe- 




in pronation. 


muscle does not 


ripheral neu- 






stand out pro mi- 




ritis (trauma- 






ne ntl y if arm is 




t i c), not in 






flexed and at- 






lead palsy. 






tempt is made by 












another to extend 












it forcibly; if mus- 












cle is atrophied 












arm is spindle- 












shaped. 








Supinato r 


Sup i nates 


Deficient supina- M u s c u 1 o- 


Fif th c er- Dise a 


brevis. 


han d when 
forearm is ex- 
tended. 


tion of hand. 


spiral. 


vical. 


above ; a 1 s o 
in peripheral 
palsies. 



INTRODUCTION— METHODS OF EXAMINATION 



23 




Fig. 13. — Motor Points on Shoulder and Arm. (Bernhardt.) 



Supinator Longus 
Extensor Rad. Long. 

Extensor Rad. Brev.— 

Extensor Digitorum Communis 

Extensor Indicis' 

Abductor Pollicis Longus - " 
Extensor Pollicis Brev: 



Dorsal Interossei I. and II. 




Extensor Carpi Ulnaris 
Supinator Brevis 



Extensor Minimi Digiti 
Extensor Indicis 



Extensor Pollicis Longus 



Abductor Minimi Digiti 
Dorsal Interossei III. and IV. 



Fig. 14.— Motor Points of Forearm and Hand. (Erb.) 



24 



THE NERVOUS DISEASES OF CHILDREN. 
Muscles of Arm, Forearm, and Hand. — Continued. 













Diseases in 


Name of 
Muscle. 


Normal 
Function. 


Symptoms of Defi- 
cient Action. 


Innervated 
by 


Represented 
in 


which Muscle is 
commonly In- 
volved. 


Exten s o r 


Extension 


Wrist cann o t be 


M usculo- 


Seventh cer- 


As before ; es- 


carpi 


and abduc- 


flexed d o r s a lly 


spiral. 


vical. 


p e c i a 1 1 y in 


radi alis 


tion of wrist 


(extended) or ab- 






neuritis. 


longus et 


the sh o r t e i 


ducted ; flattening 








brevis. 


muse le has 
pure ex t e n- 
sion action 
only. 


of forearm. (Fig. 
ii.) 








Exten- Extension anc 


Wrist canno t be 


As above. 


Seventh cer- 


As above. 


sor carp i abduction oj 


flexed dorsally or 




vical. 




ulnaris. 


wrist. 


adducted ; " drop 
wrist " is charac- 
teristic of paraly- 
sis of extensors. 








Exte n s o r 




First phalanges 


Musculo- 


Seventh cer- 


As above. 


d i g i t o- 




cannot be ex- 


spiral. 


vical. 


1 


rum com- 


Extension of 


tended nor fingers 






J 


munis. 


fi r s t pha- 


abducted ; grasp 








Exte nsor 


\ langes of all 


is weak b ecause 








indicis. 


| fingers and 


flexor muscles are 








Ext en s o r 


abduction. 


sho r t e n e d and 








minimi 


1 


cannot contr act 


• 






digiti. 


J 


forcibly. 








F 1 e x o r 


Flexion of 


Deficient flexion. 


Median 


Eighth cer- 


As above. 


carpi 


wrist and pro- 






vical. 




radialis. 


nation. 










Fie x o r 


Flexion of 


Flexion and supi- 


Ulnar 


Eighth cer- 


As above. 


carpi 


wrist and 


nation impaired. 




vical. 




ulnaris. 


supination. 










Pal m a r is 


Flexion of 


Flexion impaired ; 


Median 


Eighth cer- 


As above. 


longus. 


wrist only. 


no an o m a 1 o u s 
position of hand 
from paralysis of 
wrist as hand falls 
by its own weight; 
the flexors of 
fingers may act as 
substitutes. 




vical. 




F 1 e x o r 


Flexes second Second phalanx 


Median 


Eighth cer- 


As above. 


di gi to- 


phal a n x to- 


cannot be flexed. 




vical. 




rum sub- 


ward first. 










limis. 












F 1 e x o r 


Flexes last 


Last two phalanges 


Ulnar and 


Eighth cer- 


As above; 


d igi to- 


two phalanges 


cannot be flexed. 


Median. 


vical. 


muscle should 


rum pro- 


toward first. 








be tested with 


fundus. 










special c a re 
in ca s e s of 
traumatic in- 


Interossei 


Abduction and 


Fingers cannot be 


Ulnar, which 
also sup- 


Eighth cer- 


juries. 
As above ; o f- 


and lum- 


adduction of; 


abducte d or ad- 


vical, fir s t 


ten the first 


bricales. 


fingers if first 


du c t e d ; inte r- 


plies th i r di 


dorsal. 


muscles to be 




phalanges are 


osseous sp a c e s 


and fourth 




affected in 




extended; 


are very marked ; 


1 u m b rica- 




progre s s i v e 




flexion of first 


" Main en griffe " 


les; median 




spinal atro- 




phalanges 


due to extension 


supplies 




phies. 




and siraul- 


of first phalanges 


first two and 






• 


tane o u s ex- 


and flexion of 


somet i m es 








tension of sec- 


second and third 


third 1 u m- 








ond and third 


phalanges. (Figs. 


bricales. 








phalanges. 


17. 18.) 









INTRODUCTION— METHODS OF EXAMINATION. 



25 



Triceps (External Hea< 

Triceps (Internal Hea 
Ulnar Ner- 



Flexor Carpi Ulnari 
Flexor Profundus Digitorun 



Flexor Subl. Digitor (III k IV 



Flexor Subl. Digit. (Inde 
and Fifth Finger) 



Palmaris Bre 

Abductor Digiti Minimi 

Flexor Minimi Digiti 

Opponens Minimi Digit 



Lumbricales 




Deltoid 

Musculo -Cutaneous Nerve 

Biceps Muscle 
a Brachialis Anticus 

Median Nerve 

». Supinator Longus 

Pronator Teres 
Flexor Carpi Radialis 

Flexor Sublimis Digitorura 

Flexor Pollicis Longus 
— -.-Median Nerve 

Abductor Pollicis Bre vis 
Opponens Pollicis 

Flexor Pollicis Brevis 

Adductor Pollicis 



Fig. 15.— Motor Points on Inner Surface of Arm. (Erb.) 




Fig. 16.— Extreme Atrophy of Thenar Muscles—" Ape Hand." (Duchenne.) 



26 



THE NERVOUS DISEASES OF CHILDREN. 
Muscles of Arm, Forearm, and Hand.— Continued. 













Diseases in 


Name of 


Normal 


Symptoms of Defi- 


Innervated 


Represented 


which Muscle is 


Muscle. 


Function. 


cient action. 


by 


in 


commonly In- 
volved. 


Thenar 


Extends first 


Impairment of ex- 


M usculo- 


First dorsal. 


As before; 


muscles : 


phalanx and 


tension and ad- 


spiral. 




more especi- 


Exte n s o r 


abducts meta- 


duction ; flatten- 




ally in amyo- 


pol 1 i c i s 


carpal b one ; 


in g of ball of 






trophies and 


brevis. 


acts with ad- 
ductor polli- 
cis longus. 


thumb. 






neuritis. 


Exte n s o r 


Extends both 


Deficient extension 


M usculo- 


First dorsal. 


As above. 


pol 1 i c i s 


phalanges of 


and ad d uc t ion ; 


spiral. 






longus. 


thumb ; al so 
adduction ot 
meta c a r p al 
bone and 
backward 
movement of 
thumb. 


second phalanx is 
flex ed toward 
first. 








Abductor 


Abduction of 


Deficient ab d u c- 


Musculo- 


First dorsal. 


As above. 


pol lie is metacarpal 


t i o n of meta- 


spiral. 






longus. 


bone ; aids in 
flexion o t 
hand. 


carpal bo n e ; if 
this muscl e and 
extenso r pollicis 
br e v i s are par- 
alyzed adduction 
results. 








Abduct o r 


] 




Mu s culo- 






pol 1 i c i s 






spiral. 






brevis. 












Opponens 
polli c i s 
and outer 


Opposi tion 


No opposition Median. 


First dorsal. 


As above. 


of thumb. 


movement. 








portion of 












the flexor 












brevis. 


J 










Abduct o r 


Flex first pha- 


No flexion; if Median and 




As above. 


pol 1 i c i s 


lanx and ex- 


muscles are par- ulnar. 






br e v i s ; 


tend seco n d 


alyzed and atro- 






flexor 


phalanx (like 


phied, ape hand is 








brevis 


inte r os s e i, 


formed. (Fig. 16. ) 








and ad- 


also have an 










ductor. 


abduction 
and adduc- 
tion action. 










Flexor 


Fie x e s end 


No flexion of end 


Median. 




As above. 


pol 1 i c i s 


phalanx. 


phalanx. 








longus. 













INTRODUCTION— METHODS OF EXAMINATION, 2J 




Fig. 17. — Extreme " Main en Griffe," after Injury to Ulnar Nerve A. Subluxation of 
Hypertrophied Heads of Metacarpal Bones B. (Duchenne.) 




Fig. 18. — Slight Atrophy of Interossei, and Beginning of ''Clawed Hand. 
(Duchenne.) 



28 



THE NERVOUS DISEASES OF CHILDREN. 



Muscles of the Pelvic Girdle and Lower Extremities. 



Name of 


Normal 


Muscle. 


Function. 


Glute al 


Gluteus maxi- 


muscles. 


mus = exten- 




sion of leg at 




hip and slight 




rotation out- 




ward. 




Gluteus med- 




ius et mini- 




mus = abduc- 




tion of leg ; 




if leg is ex- 




tended also 




lateral move- 




ment of trunk. 


External 


Outward rota- 


rotators : 


tion of thigh. 


Pyri fo r- 




mis. 




Gemelli. 




Quadratus 




femoris. 




Internal 




obturator. 




Extern al 




obturator. 




Ilio-psoas. 


F 1 e x i on and 




outward rota- 




tion of thigh. 


Tensor 


Flexion and in- 


fasciae la- 


ward rotation. 


tae. 




Adduc tor 


Adduction of 


muscles. 


thigh. 


Sartorius. 


Flexion of hip 




and knee, 




and slight 




outward rota- 




tion of thigh. 


Quadri- 


Extensor of leg 


ceps fe- 


(rectus femor- 


moris. 


is also flexes 




hip). 



Symptoms of Defi- 
cient Action. 



No extension of 
thigh ; great dif- 
ficulty in climb- 
ing ; no abduction 
of thigh; waddling 
gait, exaggerated Gluteal 
movement of pel- perior. 
vis. 



Innervated 
by 



Inferior Glu- 
teal (sacral 
plexus). 



Deficient outward 
rotation; leg 
turned inward. 



1 Flexion diffi- 
cult; in bed 

I thigh cannot be 
flexe d ; diffi- 
culty rising 
from horizontal 
position. 

No adduction; 
thigh rolls out- 
ward. 

Flexion impaired ; 
acts imperfectly. 



Sacral 

(plexus 

(muscular 

branches). 



Obtur a tor 
nerve (lum- 
bar plexus) 

Crural (lum- Fourth lum- 



Represented 



Diseases in 
which Muscle is 
commonly In- 
volved. 



Fi r s t a n d In progressive 
second sa-j atrophies; 
cral seg- these muscles 
ments. are hypertro- 

phied or atro- 
phied in vari- 
ous forms of 
dys trophy; 
mye litis of 
lower cord; 
poliomyelitis ; 
traumatic in- 
juries to cord 
and cauda 
equina. 



Fifth 
bar. 



lum- As above. 



bar plexus) 



Superior 
gluteal. 

Obtu r a t o r 
nerve, great 
sciatic and 
crural. 

Crural. 



Leg cannot be ex- Crural. 

tended ; to test it 
ask patient, who is 
lying down with 
hip bent, to stretch 
out the leg ; when 
patient is sitting 
down to extend 
leg ; or try to flex 1 
leg while patient 1 
extends forcibly in 
the sitting posture 
with leg hanging 
down. 



bar. 



Third lum- 
bar. 



Third lum- 
bar. 



Fourth lum- 
bar. 



As above. 



As above. 



As above ; very 
frequent in 
poliomyelitis. 



INTRODUCTION— METHODS OF EXAMINATION. 2$ 



Sciatic Nerve 
.Biceps Femoris (Long Head 
Biceps Femoris (Short Head 

Peroneal Nerve 
Gastrocnemius (Outer Head) 



Flexor Hallucis Longus— ■ — 




Fig. 19. 



Crural Nerve 

Obturate r Nerve 

Pectinus 

Adductor Magnus 

Adductor Long. 

Crural Nerve 



Vasti Interni| 



Gluteus Maximus 



Adductor Magnus 

Semitendinosus 

Semimembranosus 



Gastrocnemius (Inner Head) 
Soleus 



Flexor Digit. Comm. Longus 
Tibial Nerve 



Tensor Fasciae latse 




Fig. 20. 



30 THE NERVOUS DISEASES OF CHILDREN. 

Muscles of the Pelvic Girdle and Lower Extremities.— Continued. 













Diseases in 


Name of 


Normal 


Symptoms of Defi- 


Innervated 


Represented 


which Muscle is 


Muscle. 


Function. 


cient Action. 


by 


in 


commonly In- 
volved. 


Biceps; 


Flexors of leg, 


Deficient flexion ; 


Sciatic. 


Fifth lum- 


As before. 


Semite n- 


and extensors 


action of quadri- 




bar. 




dinos u s 


of hip in ordi- 


ceps may cause 








and semi- 


nary walking, 


excessive ext e n - 








memb r a- 


not in climb- 


sion; in standing ; 








nosus. 


ing stairs. 


thigh is flexed to 
excess ; trunk 
moved backward. 








Gastrocne- 


Flexion and 


Deficient flexion of Internal pop- 


Fifth lum- 


Most promi- 


mius (al- 


adduction of 


foot ; heel cannot 


liteal. 


bar. 


nently affect- 


so plan- 


foot ; toes 


be raised ; cannot 






ed in dystro- 


tariusand 


point inward; 


stand on tiptoes. 






phies and in 


soleus). 


first phalang- 
es are extend- 
ed; last pha- 
1 a n g e s in 
plantar flex- 
ion. 








poliomyelitis. 


Perone us Slight flexion, 


Deficient abduc- 


Peroneal. 


First and 




longus. 


chiefly abduc- 


tion ; plantar arch 




second sa- 






tion of foot ; 


lessened; in- 




cral. 






elevates outer 


creased by con- 










margin of 


tracture. (Figs. 










foot. 


21-24) 1 flat-foot ; 
walking tiresome. 








Anterior 


Extensors o f 


Deficient exten- 


Anterior tib- F i f t h lum- 


As above, but 


tibial 


foot and toes; 


sion; "drop-foot," 


ial. k 


bar, first sa- 


particularly in 


mus c 1 e s 


tibialis ami- 


toes scrape floor ; 




cral. 


poliomyelitis ; 
peripheral 


(tibialis 


cus is also ad- 


to clear this, ex- 






ant i c u s , 


ductor; ex- 


cessive flexion at 






neuritis ; fre- 


exten s o r 


tensor digito- 


knee and hip; 






quent also in 


d ig i t o- 


r u m in ad- 


contr a c t u r e of 






peronealform 


rum, and 


dition to mov- 


flexors and pes 






of progressive 


extensor 


ing toes, also 


equinus or equi- 






muscular 


pollicis 


elevates outer 


novarus. (Figs. 






atrophy. 


longus). 


ridge of foot 
and abducts 
foot. 


11, 26, 27.) 








Poster io r 


Adduction o f 


1 








t i b i a 1 


foo t ; outer 


1 Deficient abduc- 


Posterior First and 


: 


muscle. 


margin of 


j tion or adduc- 


tibial nerve. 


second sa- 






foot becomes 


|- tion; deformities 




cral. 






convex. 


j result from de- 








Peron e u s 


Abduction o f 


ficiencies. 


Peroneal. 






brevis. 


foot. 


J 








Inteross ei 


Abduction and 


Abduction and ad- 


Posterior 






pedis et 


adduction of 


duction of t e s 


tibial. 






lumbri- 


toes ; flexors 


deficient ; paral- 








cales. 


of first pha- 


ysis of interossei ; 






- As above. 




lanx ; extend 


hyperextension of 








second and 


first phalanges'; 










third phalanx. 


second and third 
flexed (clawed 
foot). (Fig. 25.) 








Adductor ; 


Flex first pha- 


Deficient flexion of 


Posterior 


First and 




flexor 


lanx of big toe 


toes ; foot cannot 


tibial. 


second sa- 




brevis 


and extend 


be pushed off 




cral. 




and ab- 


second. 


ground easily. 








d uct o r 












hallucis. 










- 



INTRODUCTION— METHODS OF EXAMINATION 



31 




FlG. 21. — Submetatarsal Prominence. A 
Lower than Normal, and Increased 
Plantar Arch, resulting from Contract- 
ure of Peroneus Longus. 



Fig. 22. — Exhibiting, in Addition, Promi- 
nence of the Tendon of the Peroneus 
Longus F. (Duchenne.) 





Fig. 23. — Plantar Surface 
of Same Foot, exhibit- 
ing Changes due to Con- 
tracture of Peroneus 
Longus, shortening of 
Transverse Diameter A 
C, and Torsion of Foot. 



Fig. 24. —Same Foot. 
Valgus Position due to 
Same Cause. 



Fig. 25. — " Clawed Foot " from 
Atrophy of Interossei and Other 
Intrinsic Muscles of Foot. (Du- 
chenne.) 



32 



THE NERVOUS DISEASES OF CHILDREN. 




Fig. 26. — Pes Equinus in a Boy 
Five Years of Age from Atrophy 
of Tibialis Anticus. 




Fig. 27. — Pes Equinus of 
Eight Years Duration. 



Tibialis Anticus 
Extensor Digit. Commun. Longus. 



Peroneus Brevis ^ 
Extensor Hallucis Longus 



Dorsal Interossei | 




Peroneal Nerve 



Gastrocnemius (Outer Head). 
Peroneus Longus 



Soleus 



Flexor Halluci* Longus 



Extensor Digit. Comm. Bre». 
Abductor Minimi Digiti 



Fig. 28.— Motor Points. (Erb.) 



INTRODUCTION— METHODS OF EXAMINATION. 33 

Muscles of Back and Trunk. 













Diseases in 


Name of 


Normal 


Symptoms of 


Innervated 


Represented 


which Muscle is 


Muscle. 


Function. 


Deficient Action. 


by 


in 


commonly In- 
volved. 


Erector 


Extens ion of 


■ 

Lordosis of lower 


Dorsal 


S e c n d to 


In spinal dis- 


s pin as : 


lower dorsal 


spine; perpendic- 


nerves. 


12th dorsal 


eases and 


sacro- 


and lumbar 


ular line from 




segments. 


progr e s s i ve 


lumbal- 


vertebras. 


shoulder falls be- 






muscular at- 


is ; 1 n - 




ll i n d os sacrum; 






rophies. 


gissimus 




unilateral palsy 








dorsi. 




causes deflection 
of spine toward 
sound side. (Fig. 
30.) 
Lateral movements 








Quadratus 


Deflects lower 


Lumbar 




As above. 


1 umbo- 


p r ti n of 


of lower vertebras 


nerves. 






rum. 


spine lateral- 
Aid in expira- 


imperfect. 








Abdomin - 


Lordosis, with pro- 


Dorsal 


S e c n d to 


As above. 


al mus- 


tion ; also in 


trusion of nates 


nerves. 


12th dorsal 




cles. 


voiding blad- 
der and strain- 
ing at stool ; 
in k e e p i n g 
vertebras i n 
position. 


and abdomen; 
other actions defi- 
cient ; cannot 
straighten up 
f r m recumbent 
position without 
assistance of 
hands. 




segments. 





Serratus Magnus 



Transvewus Abdom 
inis 




Obliquus Abdominis } ^-:'- W"" 1 ^ W ml 

Externus ) T V^- _ \- '' i\ y' 

ffe. 



- Rect. Abdominis 



Fig. 29.— Motor Points of Trunk. (Bernhardt.) 



34 



THE NERVOUS DISEASES OF CHILDREN, 



Paralysis of individual muscles is not as frequent in the lower extremities 
as in the upper ; large groups of muscles (the anterior tibial, the posterior 
tibial) are often paralyzed together. Symmetrical palsies are common (in- 
volving thigh groups, both post-tibial groups, etc.). Complete paraplegia of 

the lower extremities is seen in 
some spinal and cerebral lesions of 
children (spastic birth palsies, dor- 
sal myelitis (poliomyelitis and my- 
elitis), and in multiple neuritis. Pa- 
ralysis may' be flaccid (spinal or 
peripheral) or spastic (cerebral, or 
spinal). If spinal and spastic, lesion 
is in lateral column. 

To test the exact extent of pa- 
ralysis, the child is to be placed on 
its back ; if it does not move the 
limbs at all voluntarily, suspicion 
of very great paralysis or of exces- 
sive pain is a safe one. 

If there is total paralysis, child 
cannot hold the leg, even if it be 
raised a few inches only; deter- 
mine whether child can abduct, ad- 
duct, flex, or extend the various 
parts. Failure to abduct may be 
due to contracture of adductors ; 
so also incomplete extension of 
knee may be due to contracture of 
flexors of knee. Contractured mus- 
cles must be considered with par- 
ticular care. In very young chil- 
dren, if it is desirable to determine 
whether there is any actual paraly- 
sis, pinch the toe steadily until the 
child draws the leg away, or may 
be expected to do so. 

With older children special tests 
can be made. Ask child to keep 
thigh flexed while you oppose ; to 
test flexors of thigh, rest your hands 
on its knee and ask it to push it away by lifting up thigh ; to test extension of 
thigh ask it to climb on a chair or observe whether it raises hip in walking. 
Place the child on a table and ask it to swing leg forward and backward ; 
to keep leg straight while the physician endeavors to flex it (for extensors of 
leg), or to keep leg flexed while physician attempts to straighten leg (for 
flexors of leg). To test flexors or extensors of foot and toes, it should be 
asked to perform dorsal and plantar flexion with and without resistance. 




Fig. 30. — Patient showing Lordosis of Verte- 
bral Column, due to Weakness of the Ex- 
tensors (Erector) of the Spine. 



INTRODUCTION— METHODS OF EXAMINATION. 35 

To raise on tiptoes, or to raise toes while keeping heel on ground are good 
tests for the posterior and anterior tibial groups. Paralysis of certain groups 
is attended by contractures of others. Deformities of the feet (pes equinus, 
equino-varus, and valgus) result from paralysis and contractures of the flex- 
ors, extensors, and of the intrinsic muscles of the feet. 

Examination of the gait is of the utmost importance ; 
an attempt should be made to have the child walk ; and 
even if it cannot walk, the position of the legs in stand- 
ing, or in attempting to walk, is often quite characteristic. 
Cross-legged position and cross-legged progression occur 
in children (spastic paraplegia or diplegia) ; the gait may be 
paretic, spastic, ataxic, or a combined form of all three. If 
the gait is simply paretic the child drags its legs carefully, 
walking slowly and without raising the feet. The spastic 
gait is common in the hemiplegias and other cerebral palsies 
of childhood ; also in spinal palsies (cervico-dorsal myelitis 
with involvement of the lateral columns). The knee is stiff, 
the leg is moved as a whole, and the child walks either on 
the ball of foot or on its toes. The ataxic gait, variously 
modified, occurs in neuritis in cerebellar disease, and in hered- 
itary ataxy. In walking the child reels, its legs are moved 
extravagantly, and it walks with a broad base. A swinging 
gait is very frequent in poliomyelitis. The gait varies accord- 
ing to the muscles affected ; in dystrophies, the gait is apt to 
be waddling, or a mixture of the waddling and swinging gait. 

To be efficient, muscular action must be well directed. The contractions 
of muscles may be entirely normal, but unless the functions of muscles act- 
ing together are properly co-ordinated, the contractions may fail of their prop- 
er effect. In all movements (in walking, standing, writing, grasping, talk- 
ing), co-ordination of the muscles therein concerned is essential. We speak 
of inco-ordination or of ataxia, if movements go wide of the mark. If a per- 
son, instead of touching the end of the nose with the tip of the fingers (eyes 
being closed), fumbles all over the face, he has ataxia of the upper extrem- 
ities ; and if instead of preserving his balance accurately in walking, a person 
sways from one side to the other, and to keep his balance at all walks with a 
broad base, that person has an ataxic gait. The ataxia may result not merely 
from inco-ordination of the muscles, but from insufficient sensory (muscular) 
impressions of the position in space of various parts of the body. If a 
person is asked to cross one knee over the other (with eyes closed), he must 
know exactly where the other knee is, if he is to perform the movement with 
nicety ; and he must be able to gauge the exact extent of muscular action to 



$6 THE NERVOUS DISEASES OF CHILDREN. 

be employed. With the assistance of vision muscular co-ordination is more 
perfect. In children ataxia is less frequent than in adults ; it occurs in mul- 
tiple neuritis, in hereditary ataxy, and in cerebellar disease. (In the last- 
named, there is considerable reeling in addition to mere ataxia ; there is 
inco-ordination and great uncertainty.) 

Before leaving the muscular system it is well to note the tonus of the 
muscles. In children this is of importance ; for by the mere handling of a 
leg the experienced physician will be able to determine whether muscles are 
normal, flabby, atrophied, or contractured. Every normal muscle, if struck 
lightly with a percussion hammer, or with a finger, will exhibit a wavelike con- 
traction. This mechanical excitability may be increased, so that the entire 
muscle contracts forcibly upon the slightest tap (tapping the quadriceps 
femoris may produce movement like that of the knee-jerk) ; or instead of 
single contractions, small fibrillary tremors may be observed on tapping 
atrophied or degenerated muscles. 

Sensory Disturbances are not easily determined in 
children. Many of the finer tests cannot be applied at all, 
for young children are not sufficiently intelligent to give 
reliable answers. The disturbances of sensation may be 
either subjective or objective. 

i. Subjective sensations may vary according to character 
of sensory perception ; thus Ave may have subjective sensa- 
tions of heat, of cold, of pressure ; if these sensations are in- 
tense, they may be equivalent to pain (Goldscheider) ; there 
may also be perverted subjective sensations, such as formi- 
cations and other paresthesias (feeling as though a part 
were swollen, enlarged, unusually heavy and the like). Pain 
may vary in character (boring, cutting, thumping, burn- 
ing) ; its description, as Goldscheider has shown, depending 
very largely upon well-known sensory impressions that 
have been associated with pain. Distribution of pain will 
vary ; it may follow the distribution of nerves (neuralgia 
and neuritis); it may be restricted to definite areas, and 
such areas of pain may hold definite relations to disease 
in distant parts (according to Head, Dana, Goldscheider, 
and others). Pain persistently referred to one spot is desig- 
nated as Topoalgia. 

2. In testing for objective disturbances of sensations, 
we must note that ordinary sensation is complex in char- 
acter; that we have perception of touch, of pain, of heat 
and cold, of the active and passive movements, of pressure 



I XTRODUCTION— METHODS OF EXAMINATION. 37 

and of resistance. There may be complete or partial loss 
of these various sense perceptions. . 

The simpler the tests the better; the sesthesiometer is 
superfluous. To determine tactile sense, passing cotton 
over the skin is generally quite sufficient ; or still better, let 
the physician close his eyes and touch the patient ever so 
lightly ; his own perception of contact will tell whether 
that of the patient is above or below his own ; asking the 
patient to state in which direction a finger is being passed 
is another good test ; a correct answer implies normal tac- 
tile perception at every point ; writing numbers on the skin 
may be tried in older children. For pain, we may test by 
pricking with a pin, by using the faradic current, by pinch- 
ing and the like. In all these tests the child's facial expres- 
sion should be studied carefully. To determine the tem- 
perature sense it is best to employ test-tubes with hot and 
cold water, or to place metallic objects on the skin and see 
whether differences in heat or cold can be made out. Gold- 
scheider has shown that there are special areas in the 
skin for the perception of pressure, of heat and of cold ; 
and that the acuteness of sensory perception varies in dif- 
ferent areas. 

His test for minimum or maximum heat and cold per- 
ceptions are not practicable, however, at the bedside — least 
of all in children. 

The term "muscular sense" led to misconceptions. It 
included a perception of passive movements, of active 
movements ; of pressure and of resistance, and a perception 
of the position of parts. 

To test the perception of passive movements it is best to take the joint 
firmly between the two hands ; then move the joint slightly and ask whether 
patient knows what has been done ; a deficiency of this sense can be made out 
easily. This test is an important one, for many cases of ataxia are depend- 
ent upon the loss of this special perception. To test perception of weight, it 
is best to use rubber balls of same size, but filled with varying quantities of 
shot. The balls are placed on the hand, on the leg, etc., and the patient is 
asked to determine which is lighter or heavier. The patient's knowledge of 
the position of parts is tested by placing one part (a leg, a finger), in a def- 
inite position, or raising it to a definite height (eyes closed) and then asking 
patient to put a symmetrical part in relatively the same position. 

Loss of sensation is termed anaesthesia ; diminished sensation, hypassthe- 



38 



THE NERVOUS DISEASES OF CHILDREN. 




Ql 

Fig. 32. Fig. 33. 

Distribution of the Sensory Nerves. (Freund. ) Figs. 31-37. 









INTRODUCTION— METHODS OF EXAMINATION. 39 




Fig. 37. 



40 THE NERVOUS DISEASES OF CHILDREN. 

sia; and increased sensory perception is termed hyperesthesia. Disturb- 
ances of sensation may vary with the distribution of sensory nerves (Figs. 
6, 3I-37)- Hemianesthesia implies loss of sensation in one half of the body. 
This is rare in children, and occurs chiefly in hysterical affections. Anoma- 
lies of temperature sense occur in some cord affections and in cases of neu- 
ritis. The stereognostic sense implies a correct perception and appreciation 
of an object through the cutaneous senses (with eyes closed the patient is to 
recognize familiar objects — such as coins, keys, buttons, placed on different 
parts of the body). Unless all sense perceptions and associated memories 
are normal, astereognosis may be developed. 

Examination of Reflexes. — We distinguish between the deep or 
tendon reflexes, and the superficial or cutaneous reflexes. The former con- 
stitute the more important class. Among the deep reflexes the patella-tendon 
reflex or knee-jerk has the greatest clinical significance ; next in order is the 
ankle clonus, or Achillis-tendon reflex, and in many cases the behavior of 
the wrist reflex, the triceps reflex, and even the jaw-jerk is carefully to be 
considered. In children it is more difficult than in adults to elicit the deep 
reflexes, for the former cannot relax their muscles easily, and thus inhibit to a 
degree every reflex movement. In testing for the knee-jerks and for other 
reflexes, it is best to take the child unawares, before it has learnt to know 
what the test means. While busying one's self with other parts of the body, 
or while talking to the child, if the leg is in a favorable position, strike the 
tendon smartly, and the reflex movement will ensue if the conditions are 
normal. If this first test does not succeed, seat the child at the edge of a 
table with its legs dangling, and then quickly strike the tendon. If the child 
continues to inhibit the reflex, I am in the habit of placing my left hand be- 
tween the crossed knees of the child in such a way as lightly to grasp the 
posterior surface of the upper leg. The child is compelled to relax its 
muscles, and I can easily tell whether it makes the slightest effort to contract 
them. If the tendon is struck, the excursion of the leg is bound to follow, 
provided the reflex is present. In older children Jendrassik's method 
(clinching the fists while the test is made) may be employed ; but it is still 
better for the physician to ask the child to squeeze his fingers the very in- 
stant he strikes the tendon. 

To elicit the ankle clonus, it is best to perform sudden dorsal flexion of 
the foot, while keeping the knee resting on the other hand and in the position 
of moderate flexion. The same clonic movements can be elicited by striking 
the Achillis tendon while the foot is in moderate dorsal flexion. Babinski 
has suggested that the patient be asked to rest the leg on a chair ; the Achil- 
lis tendon can then be struck with ease. 

The wrist reflex is tested by striking the radial side of the forearm near 
the wrist ; a smart tap will cause contraction of the supinator and biceps 
muscles. The triceps reflex is elicited by striking the tendon while the arm 
is flexed. The " jaw jerk," a reflex contraction of the masseter, is brought 
about by striking the middle of the chin when the mouth is slightly open, or 
by tapping a pencil laid on the lower teeth or jaw. I have seen this distinctly 
present in a case of multiple sclerosis in a child. 



INTRODUCTION— METHODS OF EXAMINATION. 4 1 

In testing the wrist or triceps reflexes, place the joints 
in a position of moderate flexion so as to give the muscles 
the fairest chance for contraction. The deep reflexes may 
be present, diminished or exaggerated. The knee-jerk is 
present under ordinary conditions ; the mere presence of 
the ankle clonus is abnormal ; and the reflexes in the upper 
extremity are present in some and absent in other normal 
individuals. 

The deep reflexes are diminished or absent in peripheral 
nerve disease, in spinal-cord affections involving the pos- 
terior columns and the gray matter of the cord ; in disease 
of the cerebellum. They are exaggerated in diseases of the 
brain and in diseases involving the lateral columns of the 
cord ; also in some functional diseases. 

The diminution of the reflex is determined easily enough ; 
but the absence of the reflex is more significant than a mere 
diminution. The diminution or absence of a reflex may be 
apparent only. Thus the knee-jerk may be wanting in con- 
sequence of contraction of the posterior flexors of the leg, 
or there may be anchylosis of the joint preventing free 
movement ; but in such cases the contraction of the quadri- 
ceps can be seen or felt upon tapping the tendon. In some 
children the tendon is displaced or so imbedded in fat that 
it cannot be struck readily. 

It is more difficult to state when a reflex is exaggerated. 
In this matter comparison based upon experience is the 
best guide. We can safely speak of an exaggerated reflex 
if there is an exceeding quickness of the response, if the 
slightest tapping produces a liberal movement; or if clonic 
movement results from a single blow (as in patella clonus). 
If the reflex can be elicited indirectly by striking the finger 
placed over the tendon, the reflex is surely exaggerated. 
Moreover, if a child of three years or more is so seated that 
its feet are in solid contact with the floor, and if when its 
patella tendon is struck, the foot is lifted from the floor, 
the reflex is considerably increased. 

Very lively knee-jerks do not necessarily imply organic 
disease, but exaggerated knee-jerks plus ankle clonus and 
a Babinski sign are indicative of this. Any change of the 
reflexes in one half of the body is always suspicious of 



42 THE NERVOUS DISEASES OF CHILDREN. 

organic disease, and so is the association of increased me- 
chanical excitability and of contractures with increase of 
the reflexes. 

The superficial or cutaneous reflexes are not of great importance in child- 
hood. The abdominal, epigastric, and cremasteric reflexes are but poorly 
developed in children ; the last named is absent in many older children ; and 
no significance can be attached to its presence or absence unless the condi- 
tion is not the same in both halves of the body. The plantar reflex (retrac- 
tion of the foot on irritation of the soles) deserves a few words of comment. 
Like the other cutaneous reflexes it is absent in diseases of the peripheral 
nerves, and in diseases of the spinal cord involving the course of the reflex 
arc ; if the lesion is higher than the level through which the arc passes, the 
reflex may be exaggerated, but this is not as constant a feature as is the case 
with the tendon reflexes. In all unilateral brain-lesions the cutaneous re- 
flexes are absent as a rule on the paralyzed side. The plantar reflex is gen- 
erally absent in a condition of coma (as well as in deep sleep), and its 
presence or absence may give a clew as to the degree of coma in a given case. 

The Babinski phenomenon (dorsal flexion of the big toe following irrita- 
tion of the sole of the foot) is an important symptom pointing to involve- 
ment of the pyramidal tract ; it is well, however, to bear in mind that in 
children under four years of age a dorsal flexion of the toes is the normal 
reflex. 

ELECTRICAL EXAMINATION.* 

The distribution of a paralysis, the flaccid or spastic con- 
dition of the muscles," the behavior of the reflexes, may en- 
able us to make an accurate diagnosis in many cases, but in 
many more the diagnosis cannot be safely established until 
a careful electrical examination has been made. 

The chief object of such an examination is to determine 
the response of various muscles and nerves to the faradic 
and galvanic currents, and to compare such response with 
the conditions obtaining during health. 

The younger Westphal has shown that the nerves and 

* The author cannot undertake to give a detailed account of medical electricity ; for 
this the student is referred to the treatises of de Watteville, of Beard and Rockwell, of 
Erb, Lewandowski or Toby Cohn ; also to the special chapters in the text-books of 
Gray, Dana, and Gowers. The importance of a thorough knowledge of electricity 
should not be overlooked. Whatever one may think of the value of electricity as a 
therapeutic agent, there can be no question of its great merit as an aid to diagnosis. 
Moebius argues that the experienced neurologist can make a diagnosis without the 
assistance of electrical tests, and that such tests are not wholly reliable. But they are 
reliable in fully ninety-five per cent, of all cases, and as for making a diagnosis without 
the aid of electricity, that may be possible. A clever neurologist can also diagnosticate 
a cerebral tumor often enough without the ophthalmoscope, yet he will not spurn the 
corroborative evidence which an examination of the papillae may furnish. 



" 



INTRODUCTION— METHODS OF EXAMINATION. 43 

muscles of the new-born, and of children up to the age of 
five weeks, do not respond except to very strong currents, 
and even then the contractions are slow. But after that age 
the normal nerve and muscle will respond to faradic and 
galvanic stimulation. A knowledge of the position of the 
motor points is essential. (See Figs. 7, 13, 14, 15, 19, 20, 28, 
29.) 

Always begin the examination with the faradic current, and apply the 
one pole to the nerve or muscle to be examined, and place the other pole at 
a safe distance, say on the sternum. Use mild currents (children grow very 
restless under strong currents), and determine the weakest current with which 
a contraction is to be obtained, or whether any contraction can be obtained 
at all, or not. Place the arm or leg in a position favorable for contraction 
of the muscles to be tested, and make sure that the contraction which en- 
sues is the one that should naturally follow. Do not, for instance, claim that 
the electrical conditions are normal if on applying an electrode to the ex- 
tensor digitorum communis, a flexion contraction of wrist and fingers ensues. 
And if there is any doubt as to the strength of the current, let the physician 
apply it to himself ; he will be more certain to be merciful if he does ; or if 
there is a doubt whether a nerve or muscle should respond to a given strength 
of current, let that same current be applied to the same point in the opposite 
half of the body. Thus we can compare two peronei with each other ; or we 
can compare a peroneal nerve with a facial or a median nerve of the same 
or opposite side of the body,* or we can compare the degree of excitability 
with the figures given in tabular form by Stintzing for the minimum and 
maximum currents required to produce contractions of various nerves and 
muscles. f 

A simple diminution of faradic and galvanic response of nerves and mus- 
cle occurs in light cases of peripheral neuritis, in hysterical conditions, in 
atrophies following joint disease or from disuse, and in some primary dys- 
trophies. A simple and continued increase of response is observed practi- 
cally only in cases of tetany. But in addition to mere quantitative changes 
there are also changes in the character and quality of the response to be ob- 
served in cases of degenerative disease of the peripheral nerves, of the mus- 
cles, and of parts of the spinal cord. The changes implying degenerative 
disease are referred to as the reaction of degeneration. 

*In children the motor points may be obscured by deposits of fat. 

t American faradic batteries are now provided with a sliding scale which enables the 
physician to record how far apart the primary and secondary coils were when first con- 
traction was obtained. In recording the effect of the galvanic current, the strength 
should be stated in milliamperes. 



44 



THE NERVOUS DISEASES OF CHILD REM. 



The following table will give the salient features of nor- 
mal electrical conditions and of the reaction of degenera- 
tion : 



Normal Electrical Conditions. 


Reaction of Degeneration. 




Nerve and Muscle. 


Nerve. 


Muscle. 


Faradic cur- 
rent. 

Galvanic 
current. 

Order of 
c o n t Tac- 
tions. 


Contractions good ; 
prompt. 

Contractions prompt and 
quick. 

i. K. C. C* 

2. A. C. C. 1 interchange- 

3. A. O. C. j able. 

4. K. O. C. 

5. K. C. Te. (rare). 


No response 
(except in 
partial R. 
D.). 

No response. 


No response (except in partial 
R. D.). 

Increased excitablity at first, 
then diminished ; contrac- 
tions sluggish. 

With stronger currents. 

2. K. C. C. \ A.C.OK.C.C. 

3. A. O. C. ' 

4. K. O. C. 

or 
A. C. C. = K. C. C. 



* K. C. C. = Kathodal closure contraction ; A. C. C. = Anodal closure contrac- 
tion ; A. O. C. = anodal opening contraction ; K. O. C. = Kathodal opening con- 
traction ; K. C. Te. = Kathodal closure Tetanus. 

The preceding table states that in the reaction of degeneration (R. D.) 
the nerves and muscles fail to respond to the faradic current, but that the 
muscles continue to respond to the galvanic current in altered fashion ; first 
of all, the contractions are sluggish, and this is the most important point, and 
secondly, the order of contractions is reversed ; the anodal closure contrac- 
tion can be obtained with weaker currents than are required for the kathodal 
closure contraction. 

The mistake is commonly made to suppose that there is 
no reaction of degeneration present unless the galvanic order 
of contractions is reversed ; this occurs in the majority of 
typical cases, to be sure ; but the failure of response to the 
faradic current is early evidence of a reaction of degener- 
ation, and often precedes the development of abnormal gal- 
vanic conditions. 

In first examinations the faradic test is therefore much 
the more important. 

Many recent authors (including Remak) agree further- 
more in considering the sluggishness of contraction much 
more significant of degenerative changes than the reversal 
of the normal formula. 



INTRODUCTION— METHODS OF EXAMINATION. 



45 



It should be remembered also that muscles may be so 
much atrophied as to give no contraction to the strongest 
galvanic current. 

A partial reaction of degeneration is often overlooked ; it implies a mere 
diminution of faradic excitability of nerve and muscles, but the response of 
the muscle on direct galvanic excitation is slow, and the formula may be re- 
versed. Even if the faradic response is present, it is important to make sure 
that the galvanic response is prompt, not sluggish. 

The greatest service which electricity affords us in diag- 
nosis is in differentiating between cerebral diseases on the 
one hand and certain spinal and peripheral diseases on the 
other. The ganglion cell of the anterior horn and its ana- 
logue in the cranial nerve nuclei are responsible for the nor- 
mal electrical condition of peripheral nerves and muscles. 
Lesions involving this cell, or interfering with the transmis- 
sion of impulses from it to the periphery are attended by 
changes in electrical reactions; in all other diseases the 
electrical conditions are practically unaltered. 

Whence it follows that the electrical conditions are : 



Normal in 



All cerebral diseases,* excepting those 
of cranial nerve nuclei. 

Diseases of lateral and posterior col- 
umns of spinal cord. 

Functional troubles. 

Mild peripheral troubles. 

In some forms of muscular dystrophy. 



Distinctly Altered (R. D.) in 



1. Bulbar paralysis (acute and chronic). 

2. Polioencephalitis superior (Wernicke). 

3. Poliomyelitis (acute and chronic). 

4. Progressive amyotrophies. 

5. Amyotrophic lateral sclerosis. 

6. Myelitis, but only in muscles repre- 

sented in diseased level. 

7. Gliosis and tumor involving gray mat- 

ter. 

8. Anterior root disease (syphilis, tumor, 

etc.) 

9. Vertebral disease (Pott's disease, tu- 

mor). 

:o. Peripheral neuritis (traumatic, rheu- 
matic, toxic). 

ci. In some forms of muscular dystrophy. 



* A very few exceptions have been reported. 



To complete the examination, be sure to look for 
trophic symptoms which occur in many functional disor- 
ders as well as in disease of the peripheral nerves and in 
some diseases of the spinal cord (gliosis, tumor); and finally 
the conditions of the vesical and rectal reflexes should be 



46 



THE NERVOUS DISEASES OF CHILDREN. 



recorded ; though in regard to them the influence or lack 
of previous training' of the child must be taken into ac- 
count. A sudden loss of control is often associated with 
serious cerebral and spinal disease. 

Lumbar Puncture is a recent addition to our methods 
of examination. It is practised for the purpose of with- 
drawing some of the cerebro-spinal fluid. The examination 
of this fluid may render valuable aid in the diagnosis of 
cerebral and cerebro-spinal affections. Its value as a ther- 
apeutic measure is still in doubt. 

Lumbar puncture was introduced by Quincke. His 
directions are as follows: The patient is to lie on his left 
side, near the edge of the bed, with knees well drawn up. 
The trocar is to be inserted in the median line between the 
third and fourth or between the fourth and fifth lumbar 
vertebrae (Fig. 38). After reaching the dural sac the trocar 




Fig. 38. (After Quincke.) 
Slightly Modified ; the Horizontal Lines Correspond to the Lumbar Vertebra 1 



needle is withdrawn and the cerebro-spinal fluid will flow 
out. Quincke advises connecting the canula with a small 
rubber tube and a glass manometer. If there is no ob- 
ject in measuring the pressure at the time of tapping, 
the trocar is sufficient and the fluid may be received in a 
test-tube. 



INTRODUCTION— METHODS OF EXAMINATION. 



47 



To determine the site for the puncture, draw a line at a 
tangent to the crest of the ilium ; this will strike the fourth 
lumbar space. 

General or local anaesthesia is unnecessary except in un- 
ruly patients. Do not withdraw more than 20 to 30 c. c. at 
a sitting. Use sterilized test-tubes. 




Fig. 39. (After Quincke.) 



The procedure is a harmless one except in cases of cere- 
bellar tumor and in neoplasms in the vicinity of the fourth 
ventricle. 



PART I. 

GENERAL NERVOUS DISEASES. 



CHAPTER II. 

CONVULSIONS— ECLAMPSIA INFANTUM. 

The peculiar seizures so common in infancy, and desig- 
nated as convulsions, constitute a symptom, not a form, of 
disease. The unusual frequency of convulsions in early 
childhood points to the greater excitability in the child of 
the motor mechanism of the brain. The motor centres in 
the cortex are more apt to " discharge," and the inhibitory 
power of the brain is less developed than in the adult. 
Eclampsia has been found to be a convenient term for the 
condition in which convulsions are apt to occur, but it 
should be remembered that it is neither a disease nor a 
sufficient diagnosis in any given case, for eclampsia in 
children, as well as in adults, may be the result of many 
widely different disorders. 

However frequent these convulsive seizures may be in 
early life, healthy children are as exempt from them as are 
healthy adults. If they are not the result of organic disease 
of the brain, their occurrence indicates functional involve- 
ment of the brain in connection with disease in other parts 
of the body. 

A convulsion is a motor discharge resulting in muscular 
contractions of one or more parts of the body. In the major- 
ity of cases these convulsive movements are associated with 
a number of other symptoms ; viz., an initial cry, turning 
of the eyes upward or inward, very sudden loss of conscious- 
ness, involuntary passage of urine and of fasces, prolonged 
drowsiness, and a condition of stupor or of coma. The 
muscular movements may be tonic at first, but are apt to 
lead to clonic spasms. No two convulsive seizures are 
exactly alike, but the student who has seen a single seizure 
will not forget the main features of the condition. The 



52 THE NERVOUS DISEASES OF CHILDREN. 

convulsion may come on without warning, or the child may 
have complained of uncomfortable sensations in the head or 
stomach, of a little dizziness or of nausea ; the child gives a 
shrill cry and at once is thrown into tonic and clonic con- 
vulsions. These may be partial or general, unilateral or 
bilateral. The legs are stiff, the arms bend at the elbow, 
the wrists are flexed, and the hands clenched, with the 
fingers firmly closed upon the thumb. The head is thrown 
back or rotated to one side ; the back may be arched, the 
face is pale at first, but soon reddens and the eyes remain 
wide open. The pupils do not react. In older children 
the tongue may be caught between the teeth, and bloody 




Fig. 40. — Child of One Year Photographed During a Severe Convulsive Seizure. 

froth may be noticeable at the lips. In very young chil- 
dren we have the ordinary foam at the mouth. The respi- 
ratory muscles are in a condition of spasm ; the child often 
turns blue rapidly and is in danger of asphyxia. Fortu- 
nately the tonic spasm soon ceases, slighter clonic convul- 
sions then set in, and after a little the child grows quiet, 
and is certain to lie in a dazed or stuporous condition for a 
period that may vary from several minutes to as many 
hours or days. While the clonic spasm lasts there is im- 
minent danger of injury to the child's head and limbs in 
consequence of the severe jactations, but this danger is not 
as serious as that from asphyxia during the period of tonic 
spasm. 

Causes. — Convulsions always denote " cerebral (cortical) 
irritation." It might be sufficient to state that any morbid 



CONVULSIONS— ECLAMPSIA INFANTUM. 53 

process which brings about cortical irritation, directly or 
indirectly, is liable to cause convulsions. But it will be 
better to enumerate the chief conditions under which in- 
fantile convulsions occur. 

I. Convulsions occurring within the first few days ol 
life are, as a rule, the result of meningeal hemorrhage, due 
either to protracted labor, or to instrumental delivery. If 
the child survives, the injury done to the brain often leads 
to the development of spastic palsies. (Cf. chapter on Cer- 
ebral Palsies.) 

II. The convulsions may be due to organic disease of the 
brain, such as tumor, abscess, meningitis, or vascular lesions ; 
in all of these cases they may be the earliest symptom of 
the disease, but other symptoms associated with them, such 
as headache, paralysis, optic neuritis, and the like, will be 
forthcoming. 

III. They may mark the onset of any acute infectious 
disease. The initial convulsion in children may have the same 
value as the initial rigor in the adult. The former, with or 
without fever, is the clinical equivalent of the latter. It is 
common at the onset of pneumonia, scarlet fever, and measles ; 
and I have also witnessed convulsions in malarial fevers, and 
at the onset of chicken-pox. The general practitioner is 
very apt to err in the interpretation of such convulsions. 
A child that has had a convulsion, preceded or followed by 
a rise of temperature, is often suspected to be in the first 
stages of a meningitis or of some other cerebral disease. 
While this is true in some instances, the fact is entirely 
overlooked that the seizure may be the initial symptom not 
only of any of the ordinary infectious diseases, but of the 
acute spinal diseases as well — poliomyelitis, for instance 
(probably an infectious disease). 

IV. The convulsions may be of reflex origin. Almost 
every possible peripheral disturbance has been supposed by 
one author or the other to be a sufficient cause of convul- 
sions. I need merely mention ocular insufficiencies, nasal 
obstruction, narrow prepuces, not to call up a dozen or 
more about which there has been much, and often bitter, 
discussion. Of the influence of two conditions there can be 
no reasonable doubt. The first of these is dentition ; the 



54 THE NERVOUS DISEASES OF CHILDREN. 

second, gastro-intestinal irritation. There has been some 
question whether a tooth about to cut through the gum can 
cause an eclamptic seizure. Delayed dentition is so fre- 
quently associated with rickets that the latter condition is 
considered by many to be the chief factor, and this I be- 
lieve to be true ; but convulsions do at times occur in chil- 
dren who present no tangible signs of rickets. Moreover, 
there is good reason why a cutting tooth should produce 
convulsions in a child so disposed, say by rickets, or any 
form of exhausting disease. " The cutting tooth " is a direct 
irritant to the filaments of the trigeminal nerve, which car- 
ries this irritation easily enough to the convulsive centres 
at the base of the brain. I have little doubt of this special 
reflex origin of convulsive seizures since observing in the 
adult the occurrence of severe epileptic paroxysms in cases 
of trigeminal neuralgia. 

The influence of gastro-intestinal irritation is well illus- 
trated by the convulsions occurring in the course of an 
acute or chronic intestinal catarrh, in the ordinary summer 
diarrhoea of young children, or with the exhausting chronic 
diarrhoea in older children. The same phenomena are apt 
to occur in the presence of animal parasites (pin-worms, 
and, above all, tape-worms), and I have known severe con- 
vulsions to disappear promptly after the removal of a tape- 
worm in children between the ages of four and eight years 
never to return again. 

V. Convulsions may be due to poisons (organic or me- 
tallic) circulating in the blood. Under this heading we 
may include the convulsions of uraemia, those due to the 
presence of ptomaines in the stomach and bowels, and con- 
vulsions produced by the administration of lead and other 
metallic poisons. Toxaemic convulsions are not as common 
in the child as in the adult, unless, following recent studies 
by Chenbach and others, we consider the convulsions of in- 
fectious fevers and of intestinal troubles to be due to some 
form of auto-intoxication. 

VI. Convulsions may result from severe loss of blood, 
from any exhausting disease, or from such constitutional 
disturbances as scrofula and rickets. A vast literature has 
grown up on the subject of rickets and convulsions. What- 



CONVULSIONS— ECLAMPSIA INFANTUM. 55 

ever theory one may be willing to adopt in order to ex- 
plain this close relationship, the fact remains that an enor- 
mous percentage of children having convulsions are af- 
fected with rickets. 

An hypergemic condition of the cortex analogous to the 
condition of the bones in rickets, is the only explanation we 
can give of the frequency of convulsions during rickets, 
unless we are satisfied to accept the very vague statement 
that the latter is a simple predisposing cause of convul- 
sions. 

VII. A convulsion may be idiopathic, hereditary, or, if 
you choose, the first incident in the course of an ordinary 
epilepsy. This possibility must be kept in mind, but it is a 
more probable explanation if the child happens to be above, 
rather than under, four or six years of age. This inter- 
pretation of a convulsive seizure occurring in a child should 
be given only after excluding every other possible cause. 

It is surprising, but a fact nevertheless, to which I have 
called attention a number of times, that the more carefully 
we scrutinize cases of convulsions, or of epilepsy for that 
matter, the fewer of them appear to be truly idiopathic. It 
will be well not to make the diagnosis of an incipient epi- 
lepsy unless we are informed that the child has had previous 
convulsions at somewhat long intervals, or unless we have 
occasion to observe subsequent convulsions without a dis- 
tinct additional cause for each seizure. 

VIII. Convulsions may be due to traumatism. In some 
cases actual hemorrhage over the motor centres is the im- 
mediate cause of the convulsions. In other cases no tan- 
gible injury to the brain has resulted, and we are therefore 
compelled to regard the spasm as the expression of shock. 

Theories. — Many theories have been advanced to ex- 
plain the occurrence of convulsions. No theory is entirely 
satisfactory ; but by physiological experiments and the ex- 
periment of disease we have learned to know the conditions 
under which convulsions are most apt to occur. 

There are, first of all, the time-honored experiments of 
Kussmaul and Tenner, showing that convulsions occur in a 
rabbit after suddenly tying the cervical arteries and thus 
cutting off the blood-supply. Their conclusions are op- 



56 THE NERVOUS DISEASES OF CHILDREN. 

posed to the older theories that these seizures were due to 
an hyperaemic condition of the brain. The question arises 
whether the sudden withdrawal of blood deprives parts 
of the brain of their functions, or whether it simply acts as 
an irritant to the cortical centres. The latter explanation 
seems plausible, in view of the experiments of Hitzig, Fer- 
rier, Horsley and others. A mechanical irritation of the 
pons (floor of the fourth ventricle), as proved by Nothnagel, 
also produces convulsions. It is evident that toxic agents 
have the same effect as the electrical or mechanical irrita- 
tion just referred to, else we could not explain the convul- 
sions due to toxic agents, those of uraemia, for instance, 
and those due to asphyxia from the accumulation of car- 
bonic acid in the blood. 

Every morbid process in the motor areas of the cortex, 
if not absolutely destructive, is apt to cause convulsions ; 
but what is the relation between the convulsive centres in 
the cortex and Nothnagel's epileptic centre in the pons? 
Does the cortex contain an actual convulsive centre? If 
you irritate the lower epileptic centre in an animal whose 
hemispheres have been divided from the rest of the brain 
you will get convulsive spasms of the entire body. The 
lower centres have the power, therefore, of " starting " con- 
vulsive seizures, and it is probable that they are under 
higher control in the fully developed brain, and so long as 
normal conditions exist. It is this power to control, this 
inhibitory force, that resides in higher centres. The re- 
moval of this inhibition through disease of such centres 
liberates the energy of the lower centres.* 

Age and Frequency. — By far the largest majority of 
convulsions occur during the first two years of life. The 
Philadelphia Health Reports, as condensed by Lewis, give 
ample proof of this. 

Seven thousand five hundred and eight deaths among 

* Neurologists, the world over, have adopted Hughlings Jackson's views without en- 
dorsing every detail of his theories. Jackson considers the central nervous system to 
be made up of three tiers of sensory-motor centres. All parts of the body are repre- 
sented in each tier, but the higher centre controls one or both below it, and has the 
power to inhibit the discharges of a lower centre. In a young child the higher tiers do 
not exert a controlling influence, since they are imperfectly developed ; hence the dis- 
charges are so much more frequent than in later years. 



CONVULSIONS— ECLAMPSIA INFANTUM. 



57 



minors (during period of 1 876-1 883 inclusive) were due to 
convulsions and laryngismus stridulus. Of these were : 





Under 
1 year. 


1 to 2 
years. 


2 to 5 
years. 


5 to 10 
years. 


10 to 15 
years. 


15 to 20 
years. 


Cases of convulsions .... 

Cases of laryngismus 

stridulus 


4,993 
24 


i,335 
7 


893 

9 


178 

5 


38 


26 






Total 


5,oi7 


i,342 


902 


183 


38 


26 







While the statistics illustrate a general truth, some com- 
ment is necessary. First, convulsions occurring at this 
early age are not necessarily fatal, although those occur- 
ring in the first few days after birth are much oftener fatal 
than those occurring later. The brain has less power of re- 
sistance, and the processes giving rise to these early con- 
vulsions are a more serious menace to the life of the child. 

Secondly. It should not be forgotten that convulsions are 
so much more frequent during the first two years of life 
because the diseases and conditions causing convulsions are 
most frequent at this period. 

We must take into account an injury inflicted upon the 
brain during labor, defective brain development, dentition, 
the acute infectious diseases, and almost all the infantile 
cerebral palsies and gastro-intestinal disorders. 

Convulsions may, however, occur at any age ; but since 
the child's brain grows less irritable the older it grows, a 
convulsion occurring later in life often signifies more serious 
trouble. 

The frequency of convulsive attacks in any given case 
will also depend upon the nature of the disease, of which 
it is merely a symptom. In the case of convulsions due to 
dentition, a convulsion or a series of convulsions may occur 
with the cutting of each tooth ; if due to gastro-intestinal 
irritation, convulsions may recur until the condition is im- 
proved or relieved. In acute infectious diseases we are 
apt to have but one initial convulsion, or at most two or 
three distinct attacks ; if there were more than a few con- 
vulsions during an attack of an acute infectious disease, I 
should suspect some cerebral complication. 

It is a fact, important and well worth remembering, that 



58 THE NERVOUS DISEASES OE CHILDREN. 

the initial convulsion is as little apt to be repeated as is the 
initial rigor. The first toxic invasion, and not the fever, 
seems to cause the convulsions. I have often observed 
convulsions with relatively low temperatures (103° F.) at the 
onset, without any repetition of the same during the course of 
an acute disease, even though the temperature reach 105 F. 
and over. I do not mean to deny, however, that high fever 
alone may cause convulsions, but it is not a common cause. 

I have often had occasion to remark that repeated con-, 
vulsions are characteristic of cortical disease, and this is 
borne out by the cerebral diseases of childhood. 

In the majority of such cases the convulsions will be but 
one of a series of symptoms ; and if convulsions occur re- 
peatedly without any further evidences of cerebral disease, 
the suspicion of true epilepsy must be entertained. 

Symptoms. — It may seem odd that we should treat 
of the symptomatology of convulsions, which we have 
termed a symptom and not a disease, but the sequence of 
events during a convulsion is subject to great variations, 
and on this head there is need of further remark. A con- 
vulsion may be partial or general. Partial convulsions in 
the majority of the cases denote organic disease of the brain, 
and in this sense may be more serious than general convul- 
sions ; a slight convulsive twitching of a thumb may seem 
a small matter indeed, but it may be the first sign of a cere- 
bral tumor which is bound to be fatal before long ; while, as 
regards danger to life, a severe general convulsion may be 
entirely harmless. Partial convulsions are often unilateral, 
and sometimes limited to a single member. 

The cortical centre of the part convulsed is the chief site 
of the lesion. Partial convulsions often become general ; it 
is of importance, therefore, to inquire whether convulsions 
that affect all parts of the body start in any one particular 
member, or whether the person who is subject to general 
convulsions ever had unilateral partial convulsions. 

All the phenomena of a convulsive seizure need not to be 
exhibited in each attack. We may have convulsive twitch- 
ings without loss of consciousness, and loss of conscious- 
ness without twitching movements. I have clearly in mind 
a child about three months old, whom I had reason to see 



CONVULSIONS— ECLAMPSIA INFANTUM. 59 

some nine years ago. Without any known cause the child 
was in a " faint spell," during which time the skin was pale, 
and the pulse became slow and irregular. Such a spell oc- 
curred about the same hour on successive afternoons, and 
later on the child had several each day. No other symptom 
could be made out, with the exception of a distinct enlarge- 
ment of the spleen. The attacks were evidently malarial 
and yielded quickly to large doses of quinine. The child 
has had no convulsions since. 

There is some difference of opinion among authors as 
to whether a convulsive attack begins with clonic or tonic 
movements, and which preponderate. It is well to know 
that, according to the researches of Unverricht and others, 
tonic and clonic convulsions are different in degree and not 
in kind — a tonic convulsion being practically a rapid suc- 
cession of clonic movements. In children tonic spasm is 
supposed to precede clonic spasm, and in this respect to 
differ from the epileptic paroxysm of later life ; but the dis- 
tinction is of no importance, and, moreover, I have seen 
many spasms in children which were purely clonic from 
beginning to end. 

One special form of convulsive seizure in children de- 
serves special mention. This is 

Laryngismus Stridulus. — Cerebral croup, inward con- 
vulsions, child-crowing, etc. The many synonyms are in- 
dicative of the frequency of this condition, which occurs 
more often in European countries than here, owing prob- 
ably to the greater spread of severe forms of rickets in 
England and on the Continent. 

In its simpler form laryngismus stridulus implies merely 
a spasm of the glottis. A child that may have exhibited an 
occasional crowing, croaking noise, is seized quite suddenly 
with severe spasm of the adductor muscles ; the body be- 
comes rigid, the head is drawn back, the face grows pale 
and then livid. In a few seconds the spasm relaxes, a deep 
inspiration follows with a hissing sound, and all is over for 
the time being, except that the child presents a haggard, 
tired look. Several such spasms may follow quickly upon 
one another. After the attack is over the child often 
vomits, is badly frightened, and from sheer fatigue falls 



6o THE NERVOUS DISEASES OF CHILDREN. 

into a more or less natural sleep. It is not rare to have as 
many as twenty and more of such attacks during the course 
of twenty-four hours; in other cases single attacks follow 
each other at much longer intervals. It is quite excep- 
tional, however, to have but a single spasmodic attack. 

Other convulsive actions are apt to be associated with 
the laryngeal spasm. The diaphragm and other respiratory 
muscles are often convulsed; the fingers are firmly clenched, 
and the toes are in extreme plantar flexion (" carpopedal" 
spasm); general convulsive movements, and a total loss of 
consciousness may be developed before the seizure is over, 
proving the close relationship between the laryngeal spasm 
and general convulsions.* 

Causation. — There was much dispute among the older 
authors as to the true cause of laryngeal spasm. An en- 
larged thymus gland pressing upon branches of the vagus 
was supposed to be the most frequent cause; but. this has 
been entirely disproven by the autopsies of Henoch and 
others, who showed that there was no correspondence be- 
tween the state of the thymus and the occurrence of " in- 
ternal convulsions." An overpowering weight of opinion is 
in favor of a close causal relationship between rickets and 
laryngismus stridulus. The researches of Elsasser, of West, 
Gee, Gay, Henoch, Jacobi, Heubner, and many others have 
offered substantial proof of this position. Of late years 
there has been a disposition to discredit this theory. Loos 
has denied the influence of rickets, while endeavoring to 
establish a relation between this convulsive disorder and 
tetany. To my mind the only proper relationship between 
the two is that they are both due, safely enough, to the 
influence of rickets. Loos has been severely criticised, and 
his facts have been disproven by Kassowitz, whose large 
experience of rickets has stood him in good stead. 

Rachitic softening of the skull (craniotabes) has been 
supposed by Jacobi and others to be the active cause of 
laryngeal spasm. That the association of these conditions 
is unusually frequent cannot be doubted. Kassowitz states 
that only 48 of 370 cases of laryngeal spasm did not present 

* Children suffering from laryngismus stridulus are prone to the ordinary eclamptic 
fits, thus furnishing further evidence of the close relationship between the two conditions. 



CONVULSIONS— ECLAMPSIA INFANTUM. 6 1 

marked craniotabes ; but of these 48 cases 47 presented 
some other decided symptom of rickets. The fontanelles 
were closed in only 4 of these 370 cases, although no of 
these children were over one year of age. When rickets is 
not present the fontanelle should be closed at the end of 
the first year (Kassowitz). 

But is a softened skull the direct cause of laryngeal 
spasm ? Scarcely. The centres for vocal movements are 
far away from that part of the brain which is apt to be 
compressed in cases of craniotabes. The hyperaemic condi- 
tion of the brain in rickets is of a piece with the hyperaemic 
condition of other structures and it is this hyperaemia 
which causes an unusual irritability of the centres which 
would not under ordinary circumstances " discharge " upon 
the slightest peripheral irritation (cold air, dentition, slight 
gastro- disturbances). Indeed there may be spontaneous 
discharge of these centres without peripheral irritation. 

Laryngeal spasms occur most frequently between the 
sixth and eighteenth months, the very period during which 
rickets is most marked, if present, though it occurs often 
enough up to the age of three years and later. Patients sub- 
ject to attacks are most apt to suffer from them during the 
coldest months of the year, viz., January, February, and 
March. 

Much has been made of the supposed relation between 
laryngismus stridulus and tetany. Cheadle believes the 
two to be identical. Facial contractility may be present in 
cases of laryngismus, but cases of laryngismus in which 
the Trousseau symptom (spasm induced by compression of 
the artery in a limb) can be elicited during the interval be- 
tween attacks are surely rare. 

There is another form of convulsive seizure in children 
which reminds one of laryngismus. Many years ago a child, 
aged eighteen months, apparently healthy, was brought to 
me that would hold its breath in many a crying spell ; after 
holding its breath for a few seconds it would turn blue, and 
its head would drop forward as it lost consciousness. After 
a few more seconds the child recovered consciousness, and 
all was well again until the next crying spell, when the 
same sequence of symptoms would be apt to occur. Treat- 



62 THE NERVOUS DISEASES OF CHILDREN. 

ment by bromides and small doses of chloral put an end to 
these attacks in a few weeks. 

Diagnosis of Convulsions. — It is scarcely credible 
that any one who has ever seen a convulsive seizure can 
mistake it for anything else. And yet it has happened 
within the author's experience that he was called to a case 
of supposed tetany, which was, however, nothing more than 
an ordinary convulsive attack. 

The difficulties of diagnosis that arise are concerned al- 
together with the possible etiological factor in a given case. 
The physician who witnesses a convulsive seizure will do 
well to be reserved in giving an explanation of the origin 
of such seizure until he has carefully examined into the 
previous history of the case ; and if he does this he will be 
able to say with great certainty whether the convulsion is 
due to some reflex irritation, to some cerebral or spinal dis- 
ease, or whether it is the initial symptom of an acute in- 
fectious disorder. It is of the greatest practical importance, 
however, to be able to say distinctly whether the convulsion 
is the expression of functional disease or of organic disease 
of the brain. General convulsions are, in the vast majority 
of cases, of functional origin. Partial convulsions are, as a 
rule, the result of organic disease ; but a child that has gen- 
eral convulsions may at some previous stage of its history 
have exhibited partial seizures, or a general convulsion may 
have begun in a strictly localized fashion. The part first 
convulsed, or the part alone convulsed, indicates that the 
centre in the brain which governs the movements of this 
special part of the body is the chief site of the lesion. A 
twitching movement of the thumb, so slight that many 
might be tempted to regard it as a trifling nervousness, indi- 
cates disease in or near the centre governing the movement 
of the thumb. A convulsive seizure, beginning with a 
twitching of the eyelid, with the drawing up of one corner 
of the mouth, is certain to be the result of disease in the 
respective centres of these parts. 

The gravity of a convulsive seizure will depend very 
much upon the cause of the attack. In general terms a par- 
tial epilepsy pointing to organic lesions of the brain is a more 
serious matter than a general epilepsy, which may be func- 



CONVULSIONS— ECLAMPSIA INFANTUM. 63 

tional ; but if a general convulsion is the expression of a 
typical epilepsy about to be developed, its importance is as 
grave as though it were due to gross disease of the brain. 
Convulsions occurring at the onset of acute diseases are 
much more apt to pass off without leaving a trace behind 
them than are those convulsions which occur during the 
further course of the disease. 

There is considerable difference of opinion among au- 
thors regarding the remote dangers of convulsions. Many 
are inclined to believe that convulsions are always sympto- 
matic, and that they rarely constitute a serious danger to 
the child's life ; but others, like myself, relying chiefly upon 
a careful study of infantile cerebral palsies and of epileptic 
convulsions in the adult, are confident that convulsions are 
capable of, and often do give rise to, serious disturbance. 
This is not surprising if we consider that extreme cyanosis, 
with an accompanying intense hyperaemic condition of 
the brain, occurs during the acme of the convulsion. I 
have myself recorded a case of a child dying in an epilep- 
tic convulsion, in which at the post-mortem table a wide- 
spread recent subpial hemorrhage over the convexity and 
at the base was found to have been the immediate cause of 
death. Eustace Smith refers to a case with hemorrhage 
over the base, evidently the effect of convulsions, while 
Money and others have reported cases in which a palsy, 
setting in after a convulsion, was evidently due to hemor- 
rhage from the smaller vessels of the brain coming on dur- 
ing, and caused by, a convulsive seizure. The possibility 
of such occurrences makes a convulsion a serious matter. 
It must be the earnest endeavor of the physician in attend- 
ance on the child to bring a convulsive seizure to an end as 
soon as possible. The longer a seizure lasts, the more fre- 
quently it is repeated, the greater the danger becomes — if not 
to the life of the child, at least to its future mental condition. 

Hence it follows that we must be guarded in giving a 
prognosis of the child's condition after the seizure is over, 
the majority of them leaving no unfavorable trace upon the 
child, but every now and then cases occur in which the 
convulsions were the first of a long series of disasters. I 
shall never forget the case of a bright child who had a con- 



64 THE NERVOUS DISEASES OF CHILDREN. 

vulsive seizure at the onset of chicken-pox ; a rapidly de- 
veloping idiocy was the result. Seizures occurring in the 
first few days after birth, inasmuch as they are the expres- 
sion of serious disease in the brain, give a particularly un- 
favorable prognosis. 

Treatment. — The first object of treatment should be to 
check the convulsion. Many remedies will have been ap- 
plied long before the physician has arrived at the bedside 
of the patient. Hot baths, mustard poultices over the spine, 
the abdomen, to the soles of the feet are still in vogue and 
urged not only by knowing mothers, but also by many phy- 
sicians (by Meigs and Pepper, for instance) ; but all these 
measures are to be deprecated, for they imply a loss of 
valuable time. If a convulsion ends while the child is in a 
hot bath it does so because it has run its course. 

To check the convulsion inhalations of chloroform, or of 
chloroform and nitrite of amyl in equal parts, are much to 
be preferred to other forms of medication. A few drops 
poured on a handkerchief and held to the nose of the 
child are generally sufficient to diminish the severity of the 
convulsive seizure, and the nurse or physician sitting by 
can keep up this method of administering the drug until 
the tendency to convulsions has for the time being entirely 
disappeared. Recognizing the seriousness of convulsions, 
the physician will do well to wait until all danger from 
further attacks has passed. After the acute stage is over 
small doses of morphia (gr. -^ to -J) or of opium, according 
to the age of the child, may be given ; and after the first 
day has been passed safely I should advise the discontinu- 
ance of the opiates and the substitution of small doses of 
bromide (3 to 5 grs.) several times a day, or small quantities 
of chloral hydrate (gr. v. to x.), either by mouth or in the 
form of a rectal enema. 

Absolute quiet is the first essential of treatment. If 
there is good reason to believe that the convulsive seizure 
was due to gastro-intestinal irritation, or to the presence of 
intestinal parasites, steps should be taken to purge the 
bowels thoroughly, say by the use of castor-oil, calomel, 
and the like, or by appropriate treatment for the removal 
of worms. As soon as the cause has been removed, treat- 



CONVULSIONS— ECLAMPSIA INFANTUM. 65 

ment by small doses of opium, bromide, or chloral, will be 
in order for at least a week or ten days after the occurrence 
of the seizure. 

If the convulsive attack is associated with fever, there 
is of course every reason for giving a tepid bath, with sub- 
sequent cool sponging. The reduction of the temperature 
will, to a certain extent, diminish the probability of repeated 
attacks. Should a child be seized with convulsions while 
in the bath, it should be removed at once and made to in- 
hale chloroform and nitrite of amyl. It is far better in 
the cases of gastric disturbance to evacuate the stomach 
through the bowels than to attempt to give emetics, which 
of course cannot be readily swallowed, or to give hypo- 
dermic injections of morphia as suggested by Steffen. For 
to cause a child to vomit that has a tendency to convulsions 
is to increase the danger to which it is exposed. 

Cases of laryngismus stridulus can be treated on very 
much the same principle as convulsions, excepting that the 
child should not be allowed to remain in the recumbent 
position ; it should be carried about, and if breathing is inter- 
fered with, forcible means, such as pulling forward of the 
tongue, slapping of the chest with cold and wet towels, 
should be resorted to in order to bring about regular, 
rhythmical breathing. 

If the convulsions are due to rickets, very active treat- 
ment, looking toward the improved nutrition of the child, 
will be necessary. It should be placed upon the albumi- 
noids, and only small quantities of the cereals allowed. 
Moreover, in these cases, Kassowitz's plan of administering 
phosphorus with cod-liver oil, as .in the scrofulous diathesis, 
is of greatest importance. Syrup of the iodide of iron, or 
any of the many preparations of iron now to be obtained in 
the market, can be administered. If the attack is the be- 
ginning of an epilepsy, no time should be lost in placing 
the child upon the regular treatment of this condition, for 
the details of which the. student is referred to the chapter 
on Epilepsy. In the cases of ursemic and other toxic con- 
vulsions, the treatment must be in accordance with the 
nature of the cause, and the condition is to be treated in 
every respect as the same condition would be treated if 



66 THE NERVOUS DISEASES OF CHILDREN. 

it occurred in the adult. If the attack is supposed to be 
due to cutting teeth, the question arises whether incision of 
the gums is apt to be of any benefit. There has been much 
discussion on this point, and yet no definite conclusion has 
been reached ; and no one, so far as I have been able to 
learn, has proved that cutting the gums has ever injured 
the child or impaired its future health. It is well enough, 
therefore, to try this method, and personally I have* little 
doubt that a temporary relief of the hyperaemic condition 
of the gums is thus afforded. That the mere incision of 
the gum, as Henoch and Lewis suggest, is apt to increase 
the peripheral irritation, I can scarcely credit, if the incision 
be made according to modern surgical practice. 






CHAPTER III. 

EPILEPSY. 

Few diseases have given rise to so much discussion 
as has epilepsy, the " morbus sacer " of ancient writers. 
Opinions regarding its cause and pathology have under- 
gone many radical changes. The causes of epilepsy were 
by most writers thought to be obscure, and it is only with- 
in very recent years that a little more light has been shed 
upon the morbid processes underlying epilepsy. Some 
claim that epilepsy is never a disease per se, always a 
symptom, while others are inclined to regard true idio- 
pathic epilepsy as a form of hereditary disease. 

Whether epilepsy be merely a symptom or a distinct 
disease, the term may be used to describe a state in which 
convulsive seizures occur at varying intervals of time, and 
in which there is no special cause for each seizure. Thus 
a child may have a convulsive fit with the cutting of each 
tooth, yet we cannot say that it has epilepsy ; but if that 
same child, after dentition and its attendant troubles have 
passed, continues to have fits at shorter or longer intervals, 
the child is surely a subject of epilepsy. Fortunately, how- 
ever, only a very small proportion of those who have con- 
vulsions during childhood develop true epilepsy in later 
years. Webber reports that of 160 cases of epilepsy only 
24 began before the age of five. 

The Symptoms of epilepsy vary with the character 
of the attacks. Unfortunately the intervals between the 
attacks are not marked by as distinct a series of symp- 
toms as is the case in tetany. In epilepsy the only symp- 
toms that we can find in the interval are the effects of biting 
the tongue, the acne resulting from the bromide treatment, 
and the general stupor and indifference which are as often 
due to the drugs administered as to the disease itself. 



68 THE NERVOUS DISEASES OF CHILDREN. 

Epileptic attacks can be divided into three classes : 
i. Major attacks, or "grand mal" 

2. Minor attacks, or "petit mal." 

3. Epileptic equivalents. 

Some patients are subject to major and minor attacks, 
and there is every possible gradation between the graver 
and lesser seizures. 

The " grand mal " attacks differ but very little from the 
convulsive seizures as described in the preceding chapter. 
It will be sufficient in this connection to enumerate in the 
order of their importance and of their occurrence the chief 
symptoms of a major attack of epilepsy. 

1. Prodromata, generally of a sensory character. At 
times there is a vasomotor or psychic disturbance. 

2. Initial cry. 

3. Loss of consciousness (very sudden). 

4. Pupils dilated ; no reaction ; eyes open or closed. 

5. Tonic, then clonic spasm of muscles (unilateral, partial, 
or general). 

6. Spasm of respiratory muscles, which may lead to 
asphyxia. 

7. Spasm of the muscles of the jaw (biting of the tongue, 
bloody foam). 

8. Spasm relaxes, movements become clonic and then 
intermittent. 

9. Involuntary passage of urine or of faeces. 

10. Gradual recovery of consciousness, followed by a 
prolonged stupor or profound sleep. 

11. The entire seizure lasts only a few minutes. 

12. The deep reflexes are diminished or increased. The 
Babinski sign may be present. 

In " grand mal " attacks most of these symptoms will be 
present, but occasionally we meet with major attacks in 
which there are no distinct prodromata ; in which the initial 
cry is wanting ; in which there is no biting of the tongue ; 
no relaxation of the sphincters. The most constant symp- 
toms are the loss of consciousness, the dilatation of the pupils, 
the spasm of the muscles, and the stupor or sleep after the 
convulsive movements have ceased. The diagnosis should 
rest upon the presence of at least two of these symptoms. 






EPILEPSY. 69 

The prodromata are of great importance. In a fair pro- 
portion of cases the patient feels that an attack is coming 
on. A vague sensation at the stomach, a feeling of numb- 
ness or of tingling in any of the extremities, are by far the 
most frequent warning signs. In other cases the aura con- 
sists of slight twitching movements, of an altered psychic 
condition, varying from a general restlessness, or irritability 
of temper, to actual maniacal excitement. In many instan- 
ces the character of the aura points to the involvement of a 
definite portion of the brain, and this is true even of cases of 
general epilepsy which are not supposed to be due to any 
gross cerebral lesion. In not a few cases temporary aphasia 
marks the coming on of an attack. 

Auditory symptoms in the prodromal stage are not un- 
common. In a little boy whom I described in a paper pub- 
lished some years ago, every epileptic attack was preceded 
by a hissing noise like that of a steam-engine letting off 
steam. A young lady, aged twenty-five, a school-teacher, 
who was subject to epileptic attacks at every menstrual 
period, is in awe of an old woman whom she sees regularly 
before each epileptic seizure. The vision of a ball of fire, 
of the colors of the rainbow, of a sudden change in the size 
of objects, is a common premonitory condition. Still an- 
other patient perceives a very foul odor during the aura ; 
but I have never met with a case of epilepsy in which a 
pleasant or unpleasant taste preceded an epileptic fit. This 
would seem to show that the sense of taste is not inde- 
pendent of the sense of smell, although some authors refer 
in a vague manner to a gustatory aura. 

Patients who have distinct aurae are on the whole 
more fortunate than those who have not, for they are able 
to prepare themselves for the attack. They can secure 
themselves against injury during the attack and may have 
time to apply remedies which either inhibit or shorten the 
spells. 

The convulsive movements should be carefully studied 
in each case, for they help to define the character of the 
epilepsy. Generally speaking, universal convulsions point 
to hereditary epilepsy. Partial or unilateral epilepsy is 
indicative of organic cerebral disease. This distinction 



70 THE NERVOUS DISEASES OF CHILDREN. 

would be a very easy one were it not for • the fact that 
partial epilepsy may at any stage of the disease, and at any 
stage of an attack, become general, so that after the lapse 
of time the convulsions due to organic disease of the brain 
can in nowise be distinguished from those which are pre- 
sumably hereditary and idiopathic. It is of the utmost im- 
portance, therefore, in making the diagnosis of epilepsy, to 
inquire into the past history of the case, and to determine, 
either by personal observation or by close questioning, the 
exact manner of onset of each attack. However rapidly 
the attack may become generalized, if it begins each time 
with distinct twitchings of the thumb, of the wrist, even of 
the eyelid, there is good reason to remove such a case from 
the category of hereditary epilepsy and to range it with 
those due to organic lesions. The attacks may come on 
at definite hours of the day or night. In " nocturnal " epi- 
lepsy an abrasion of the tongue, involuntary micturition, a 
tired feeling or a headache in the morning may be the only 
evidences of an attack.* 

The typical u minor " attacks consist of a very transitory 
loss of consciousness, without any muscular twitchings, 
without the peculiar cry, and without the involuntary pas- 
sage of urine and fasces. The loss of consciousness is often 
so slight that the child is supposed to have been "absent- 
minded " or merely " faint." Mothers and physicians, 
euphemistically inclined, are apt to speak of these attacks 
as " fainting spells." There is every reason to suspect epi- 
lepsy in any case in which " fainting spells " occur and re- 
cur without sufficient exciting cause. In some instances a 
sudden lull in the conversation of the child, a momentary 
stare, an unexpected stop when the child is practising upon 
an instrument, are the signs of petit mal ; a change in the 
child's mental condition, in its character and temperament 
(there is often increased irritability) and sometimes a mild 
form of mania, reveal the serious character of what was 
supposed to be a mere fainting spell. 

Bourneville has found a slight elevation of temperature 
during major attacks (not more than i° F.); in the status epi- 
lepticus, the condition of continuous spasms, the tempera- 

* For further details see description of convulsive attack. 



EPILEPSY. J I 

ture may rise several degrees. Thomsen and Oppenheim 
have proved the existence of a concentric limitation of 
vision, and a diminution of general sensibility for some time 
after an epileptic attack. 

The term " procursive epilepsy " has been applied to 
attacks in which the patient is suddenly impelled to run 
some distance forward, or backward ; this may be a pro- 
dromal symptom, or it may be the only manifestation of an 
attack. This variety is very rare indeed. 

We have alluded above to the existence of epileptic 
equivalents. Instead of having a typical convulsive seizure, 
the child passes into a state of mental confusion in which it 
becomes entirely irresponsible for, and unconscious of, its 
actions ; it may also pass into a condition of trance or into 
an acute mania. Prolonged periods of double conscious- 
ness are rare in children ; nor have I seen in very young 
persons the condition of narcolepsy (sudden falling asleep) 
which has been described as an occasional symptom of 
epilepsy in the adult. These " psychic equivalents " may 
be the beginning of a post-epileptic insanity. 

The mental derangement following epileptic attacks is 
often marked by violent delirium. Under the influence of 
delusions and hallucinations, the patient may be impelled 
to deeds of cruelty. Such derangement may last for a few 
hours or weeks, or it may become chronic and lead to de- 
mentia. 

Causes. — In considering the causes of epilepsy we shall 
take up, first, causes of the disease itself, and secondly, the 
causes which lead to individual attacks. That epilepsy is 
often a sad heritage can scarcely be doubted if we regard 
the statistics collected by Gowers, who finds a marked pre- 
disposition in two-thirds of 1,450 cases. The inheritance 
seemed to him to come more freely from the mother's than 
from the father's side. It is also beyond dispute that epi- 
lepsy in the ancestry is not the only predisposing cause. 
Other chronic nervous disorders have a powerful influence 
in this respect. I have known children of extremely hys- 
terical, neurasthenic mothers to develop idiopathic epilepsy. 
Chorea in the mother is very apt to lead to the develop- 
ment of true epilepsy in the child ; and, on the other hand. 



72 THE NERVOUS DISEASES OF CHILDREN. 

syphilis and alcoholism in the father exert a very powerful 
influence in this same direction.* 

According to Mendel's careful studies the majority of 
cases of hereditary epilepsy begin before the age of twenty 
years; but up to the age of forty years idiopathic epilepsy 
may manifest itself. The same author proposes to call the 
disease "late epilepsy," if it is developed after the age of 
forty years ; in twenty-five per cent, of these " late " cases 
Mendel found distinct hereditary influences. 

Accepting the truth of all these statements, I wish to 
impress one fact upon the mind of the student. Cases of 
hereditary (idiopathic) epilepsy are not nearly so frequent 
as they are supposed to be. If we examine carefully into 
the early history of our cases we shall find frequently 
that the child has either sustained some severe injury to 
the brain, or has acquired some cerebral lesion early in life. 
The paralysis and other symptoms which were due to the 
same lesion may have disappeared, but the epilepsy re- 
mains. I was led to this conclusion by my studies of infan- 
tile cerebral palsies, and my views have been quoted ap- 
provingly by various authors (among them Freud) who 
have become interested in this subject. 

A very striking instance illustrative of this is that of a 
girl, about fifteen years old, who had been treated by many 
physicians for idiopathic epilepsy without any favorable 
result. On inquiring into the early history of the case I 
was told that several years previously the girl had suffered 
a slight paralytic stroke, every vestige of which had disap- 
peared with the exception of an increase of the reflexes on 
one side of the body. Testing carefully for a possible dim- 
inution of power, I found distinct traces of an old hemi- 
plegia. What was supposed to have been a case of heredi- 
tary epilepsy was clearly a case of epilepsy due to former 
cerebral disease. 

The number of cases of idiopathic epilepsy will be still 
further diminished if we exclude from the number those 
cases in which there is evidence of defective general devel- 

* Kowalewsky has insisted on the importance of syphilis in hereditary epilepsy, 
and has made a fanciful distinction between epilepsy due to paternal or maternal 
syphilis. His statements need corroboration. 



EPILEPSY. 73 

opment of the brain; and those associated with infantile 
cerebral palsies; for the paralysis and the epilepsy are due 
to organic disease of the brain. The cases in which epi- 
lepsy is developed after an acute infectious disease cannot 
be considered " idiopathic." 

The most characteristic cases of idiopathic epilepsy are 
those in which the disease is first noticed between the tenth 
and twentieth years. A few may come on between the 
twentieth and thirtieth, but those that occur later in life, or 
in the first few years of life, should be very carefully anal- 
yzed before the diagnosis of hereditary epilepsy is made. 

It must be remembered that even in cases with dis- 
tinct hereditary predisposition some other exciting cause 
must be present to develop the disease ; thus we find that 
masturbation, disturbances of menstruation, sexual excesses, 
great emotional excitement, a blow to the head, are causes 
which lead to the development of epilepsy in those predis- 
posed.* 

Causes which Lead to Individual Attacks. — In a 
patient suffering from epilepsy any interference or disturb- 
ance of the general health of the patient may bring on an 
epileptic attack. Indigestion is perhaps the most powerful 
of these causes. Herter and Smith, in an excellent research 
on this subject, are inclined to look upon putrefactive proc- 
esses in the intestine as a frequent exciting cause of epi- 
lepsy. They do not believe that this cause would be suffi- 
cient to produce epilepsy in a person not predisposed 
thereto. The same may be said of eye strain,t of urethral 

* Reflex epilepsies are described by many authors as due to peripheral injuries 
(hand or foot) ; in such cases the aura begins with symptoms referable to the injured 
part. A permanent epilepsy is rarely established in this way, and I prefer to speak 
of reflex convulsions rather than of reflex epilepsy. 

t Ranney's contributions to this subject are put forth very earnestly, but he fails to 
prove more than that the eye-conditions (errors of refraction, muscular insufficiencies, 
etc.), may cause single attacks ; but they are surely not the cause of the epilepsy. 
Moreover, every neurologist knows that by treatment directed to the improvement of 
the eye-condition the attacks may be suspended temporarily, but the disease returns 
after a varying lapse of time. Ranney's oases are for the most part reported altogether 
too early. Hern and Dodd have given but little support to Ranney's (or rather Stevens's 
views), and their statistics are open to the same criticism. Instead of reporting the 
'• cures " it would be valuable to publish the later history of all cases operated upon. 
In 1904 the author has again reviewed this subject and has seen no reason to change 
his views. 



74 THE NERVOUS DISEASES OF CHILDREN. 

stricture, of narrow prepuces, and of laryngeal irritation. 
I doubt whether any one of these conditions has ever been 
the sole cause of epilepsy, though I am willing to concede 
that they may be sufficient to produce occasional attacks in 
persons with this special hereditary taint. 

Improper oxygenation of the blood is another frequent 
cause of attacks. This explains why so many epileptics 
have their seizures in crowded court-rooms, in theatres, and 
in poorly ventilated bedrooms. The irritation of the epi- 
leptic centres in the lower portion of the brain is directly 
responsible for these attacks. After allowing for all these 
various causes we are compelled to admit that many of the 
epileptic seizures occur at more or less regular intervals, 
for reasons that we cannot discern, though I am firmly 
convinced that the more carefully we regulate the life and 
habits of the epileptic patient the fewer attacks he will 
have. It is very largely a question of removing all excit- 
ing causes. 

A few powerful drugs have been known to cause epi- 
lepsy. Heiman described a number of cases of epilepsy due 
to poisoning by cocaine, and Tuczek has dwelt upon the 
potent influence of antipyrin. In the case of children 
cocaine need not be feared ; but the careless exhibition 
of antipyrin might be responsible for unpleasant occur- 
rences. 

Gray has called attention to the frequent occurrence 
of epileptic attacks in those suffering from chorea and mi- 
graine. I have seen several patients, in advanced years, 
in whom the epilepsy followed upon the cessation of mi- 
graine. 

Diagnosis. — The diagnosis of epilepsy offers but little 
difficulty if the character of the individual attacks has been 
clearly made out, and if such attacks recur at more or less 
regular intervals. 

The epileptic nature of a seizure is determined by the 
presence of some of the several symptoms which go to make 
up a complete attack. The chief difficulty will be experi- 
enced in differentiating between an epileptic seizure and an 
ordinary fainting spell ; also between epilepsy and hysteri- 
cal attacks. 



EPILEPSY. 



75 



The characteristic features of each attack will be found 
in the following tables : 



Epileptic Attacks. 

Loss of consciousness very sudden. 
Warning of short duration. 

Pupils dilated ; do not contract to 

light. 
Pulse unaltered. 
Tonic and clonic spasms in various 

parts of the body. 
Bloody foam at mouth. 
Involuntary passage of urine and 

faeces. 
Prolonged stupor after attack. 



Fainting Spells. 

Loss of consciousness gradual. 
Warning of some minutes before 

consciousness is lost. 
Pupils contracted or unaltered. 

Pulse feeble. 
No spasms. 

No evidence of biting of the tongue. 
No involuntary passage of urine or 

faeces excepting in rare instances. 
Recovery prompt after attack. 



Epileptic Attacks. 

Loss of consciousness sudden and 

absolute. 
Warning of short duration. 

Pupils dilated. 

Tonic and clonic spasm. 



Eyes turned upward and inward. 

Involuntary passage of urine and 

faeces. 
Prolonged stupor. 



Attacks at rarer intervals. 
Duration of attack short. 



Hysterical Attacks. 
Loss of consciousness not absolute. 

The attack often preceded by emo- 
tional excitement. 

Pupils not dilated. 

Tonic rigidity ; exaggerated conscious 
movements ; arching of back ; ex- 
cessive noises. 

Eyes staring, not turned, sometimes 
closed. 

No involuntary passage of urine or 
faeces. 

Recovery gradual ; no stupor. The 
patient may pass, however, into a 
trance condition. 

Attacks may be frequently repeated. 

Duration of attack much longer. 



The distinction between organic and idiopathic epilepsy 
can easily be made if we simply call to mind that organic 
epilepsies are, as a rule, partial, if not unilateral. Idio- 
pathic epilepsies are invariably bilateral or general in their 
manifestations. 

If organic disease is suspected, the manner of onset of the 
individual attack should be carefully determined, for partial 



j6 THE NERVOUS DISEASES OF CHILDREN. 

or unilateral manifestations often become general during 
the attack. Under the head of organic epilepsies we must 
include post-hemiplegic epilepsy. This form comes on with 
deplorable frequency after the paralytic attacks in early 
life. The epileptic movements as a rule affect the paral- 
yzed side, but after the disease has lasted for some years 
the child, although its paralysis is unilateral, is prone to 
have general epileptic attacks. Under these circumstances 
the relation between the general epilepsy and the hemi- 
plegic form of paralysis can scarcely be doubted. It will 
not be amiss to insist again upon the fact that the evidence 
of an existing hemiplegia may be so slight that it will be 
overlooked unless specially examined for; but however 
slight such traces may be, if it can be shown that the epi- 
lepsy was developed after the onset of the hemiplegia there 
is good reason to suppose that both the paralysis and the 
epilepsy are due to the same organic lesion. I consider it 
a safe rule in epilepsy beginning in childhood to examine 
particularly for exaggeration of the reflexes ; and an in- 
crease of the reflexes in one-half of the body is quite as safe 
a sign of a preceding hemiplegia as a marked paralysis with 
contracture would be. 

The sudden onset of epileptic movements in a child pre- 
viously healthy should lead one to suspect the possibility of 
intra-cranial tumor ; and a slight weakness of the parts con- 
vulsed, a possible increase of the deep reflexes in that same 
part, the presence of headaches and the development of 
optic neuritis, are the symptoms that we must look for in 
order to establish or to discard the diagnosis of tumor. 

The same series of symptoms may occasionally be pres- 
ent in cases of acquired or hereditary syphilis ; if so they 
are the result of general specific infiltration of the brain 
coverings, or of the development of gumma together with 
this general infiltration. 

Prognosis. — The prognosis of epilepsy, whether of the 
organic or idiopathic order, is grave, not hopeless. Some 
patients with undoubted epilepsy have been cured. In 
many others, do what we will, the attacks recur as soon as 
treatment is abandoned. Painstaking treatment meets its 
due reward. The author's records contain the histories of 



EPILEPSY. 7 7 

young men and women who were sorely afflicted in early 
life but have had no recurrence of epilepsy for periods 
varying from three to fifteen years. The attacks may be 
inhibited for a year and even for two years, but may return 
after that period of time if the customary treatment has 
been stopped. In this respect there does not seem to be 
a wide difference between organic and idiopathic epilepsy, 
except that in the former, malignant disease may lead to 
an early fatal termination. The frequent development of 
dementia, idiocy, or of epileptic insanity, makes the out- 
look gloomy enough in many instances. In spite of all 
recent therapeutic efforts we have not been able, however, 
to accomplish as much as is desirable in the way of a rad- 
ical cure of the disease. The most encouraging hope that 
we have is that in the beginning of the disease the attacks 
may be due to special conditions which do not necessarily 
imply the existence of hereditary epilepsy. The fond hope 
that the child may outgrow the tendency to epileptic seiz- 
ures is realized every now and then. 

Pathological Anatomy. — Our knowledge of the mor- 
bid changes in epilepsy is very incomplete. Meynert laid 
great stress upon induration of the cornu amonis. Chronic 
hydrocephalus has been found in many cases, but this can 
hardly be considered a cause of epilepsy, for if it signifies 
anything it merely implies that the same process which led 
to the development of hydrocephalus also caused the epi- 
lepsy. 

It is much more probable that the morbid changes of 
true epilepsy will be revealed through a study of the corti- 
cal changes which give rise to a localized epilepsy in cases 
of serious injury to the brain, and of epilepsy associated 
with infantile hemiplegia. In these conditions the initial 
stages of a morbid process* leading to epilepsy can best be 
studied ; and it is possible, if not probable, that general epi- 
lepsy has such a beginning at a time and in a part of the 
brain unknown to us. Marie, Fere and Chaslin have led the 
way in these studies, and report the discovery of a neuroglia 

* For other morbid processes associated with epilepsy see chapters on Cerebral Pal- 
sies and on Idiocy. 



78 



THE NERVOUS DISEASES OF CHILDREN. 



sclerosis which they consider to be the cause of epilepsy — 
such sclerosis starting from a focus of disease. These find- 
ings have been confirmed more recently by Alzheimer. 

Van Gieson examined corti- 
cal tissue, removed by McBur- 
ney, from two cases of Starr. 
He demonstrated most satis- 
factorily the changes in the 
large pyramidal cells of the cor- 
tex and in the neuroglia. He 
found that " the ganglion cells 
are affected by a series of de* 
generative changes, which in 
their most advanced stages re- 
sult in an almost complete dis- 
solution of the cell, and yet this 
degeneration is not extensive 
enough to involve the cells so 
universally as to interfere with their topographical distri- 
bution." 

Some of the degenerative changes of the cells will be 

evident in the figure below taken from Van Gieson's article. 

There is a decided change — a true hyperplasia — in the 







Fig. 41. — Diffuse Neuroglia Sclerosis 
of the Cortex in Epilepsy (after 
Chaslin). 




Fig. 42.— Various Phases of the Earlier Stages of the Degeneration of the Ganglion 
Cells. The thin lines enclosing the cells w and u represent the pericellular spaces ; 
the cells x and y show the earliest stages, w and s later stages, and k shows the 
ultimate destruction of the whole of the ganglion-cell body, leaving nothing but the 
nucleus lying in an empty space. (Van Gieson.) 



EPILEPSY. 



79 



neuroglia tissue ; clusters of an increased number of very 
young and seemingly proliferating neuroglia cells are vis- 
ible in the vicinity of the small pyramids. 

These findings were very much the same in two cases : 
in one the condition was due to a foreign body, and in the 
other an old cicatrix seems to have induced a similar path- 
ological state in the neighboring tissue. To appreciate 
such researches at their true worth, it is important to note 
that they have reference to the early conditions only. What 
the nature of the secondary changes is, and how these are 















Fig. 43. — A Group of More Mature Neuroglia Cells in the Layer of Small Pyramids. 

(Van Gieson. ) 

developed from the original forms of disease is a subject for 
further study. Sailer has demonstrated the occurrence of 
an hypertrophic nodular gliosis in cases of epilepsy associ- 
ated with dementia. Voisin questions whether this condi- 
tion will be found in genuine epilepsy. 

Pathology. — The pathology of the epileptic attack is 
identical with that of convulsive seizures in general. It is 
not necessary therefore to repeat in this chapter what was 
said in the chapter on convulsions. The only question of 
importance is whether in the diseased brain the convulsive 
centre on the floor of the fourth ventricle plays as impor- 
tant a role as it does in physiological experiments. Bins- 
wanger found that electrical stimulation of the lower half 
of the floor of the fourth ventricle in animals would pro- 
duce tetanoid attacks or spasm in the limbs and the trunk, 
but he did not succeed in causing an ordinary epileptic at- 



80 THE NERVOUS DISEASES OF CHILDREN. 

tack. There can be very little doubt that in man an epi- 
leptic attack is in the vast majority of cases the result of 
cortical irritation. The character of the sensory auras is 
evidence of this, and so is the observation made by Oebeke 
and Gowers, that epileptic attacks cease after a lesion in 
the internal capsule.* 

As for the pathology of the disease itself, opinions have 
undergone a very marked change within the last few years, 
owing to the rather startling suggestion of Pierre Marie, 
that epilepsy is often of infectious origin. Haig, Voisin, 
Fere and others advocate the theory of auto-intoxication, 
believing this to be due to the retention of toxic substances 
in the blood. Urine voided after an attack has been found 
to be more toxic than urine voided before the attack. Let 
us not forget that a focus of disease, due to purely me- 
chanical causes, may be the beginning of general changes 
throughout the brain, which are responsible for the per- 
manent establishment of epilepsy. 

Treatment. — In discussing the treatment of epilepsy it 
is best to proceed on the theory that the attack is due to 
over-action of the cortical cells. Gowers explains the influ- 
ence of the bromides by stating that " if we regard the 
morbid state in epilepsy as an instability in the resistance 
of the nerve-cells, it seems probable that the effect of the 
bromide is to increase the stability of that resistance." 
Whatever the explanation may be, we have up to this time 
found no drugs that can in any sense be considered proper 
substitutes for the bromides.f 

It is certain that very few cases of epilepsy have been 
permanently cured by the administration of the bromides ; 
but unquestionably they serve an admirable purpose in 
checking the number of attacks and in diminishing their 

* Horsley pleads strongly for the invariable cortical origin of the epileptic attack, 
and believes that during the attack the cortex is in a congested rather than an anaemic 
condition. It will help us very little to say that the attacks are due- to toxine poi- 
soning. This may or may not be true ; but in every case the most important ques- 
tion to settle is, why should certain individuals be prone to attacks and others not, 
while these same toxines from intestinal putrefaction, for instance, are present in 
hundreds of others who never have an epileptic seizure. 

t A recent writer explains the action of the bromides by their power to diminish 
the effect of the toxines circulating in the body. If toxines and bromides were in- 
jected into the veins of an animal at one and the same time, the result was less 
severe than if the toxine alone was injected. 



EPILEPSY. 8 1 

severity. To accomplish this end the bromide salts should 
be administered according to a definite plan. It has been 
my practice to give preference to the bromide of sodium, 
which I employ, according to the age of the patient, in ten 
or fifteen grain doses, three times a day. If given in a 
wineglassful of (alkaline) water after meals, the gastric 
functions will not be seriously impaired. Erlenmeyer sug- 
gested, some years ago, that a combination of the three 
salts — the bromide of sodium, the bromide of ammonium, 
and the bromide of potassium — would answer far better 
than the exhibition of any one of these salts singly. He 
went so far as to claim that if a single salt had produced 
acne, that that acne would disappear upon the administra- 
tion of the three salts combined. I have given this method 
a fair trial, but have not been able to convince myself of 
the truth of Erlenmeyer's claim. The bromides should be 
pushed in every case to the point of tolerance, and until 
the attacks have been successfully diminished or inhibited. 
The loss of the palate reflex is evidence that the patient 
has become thoroughly brominized, but in children intol- 
erance to the drug is very apt to be established long before 
this point has been reached. 

The bromides can be administered in divided doses, two 
or three times a day ; but if there is no good reason to 
give the drug during the day, there is a special advantage 
in giving the entire daily dose shortly before bedtime. The 
stupefying effect of the drug is not felt as distinctly as 
when it is administered during the daytime, and the sound 
sleep that ensues is an additional advantage to the patient. 
In the case of nocturnal attacks the medicine should be 
given before going to bed, and at no other time. In his 
various admirable lectures on the treatment of certain func- 
tional neuroses Seguin advocated the plan of giving the 
bromides on very much the same principle that one would 
adopt in giving quinine in malaria. In other words, the 
bromides should be given according to the periodicity of 
the attacks, giving little in the interval and increasing the 
dose very much at the time when an attack is expected ; or 
if the attacks are" frequently repeated, to give the drug four 
to five hours prior to the time at which the seizures occur. 



82 THE NERVOUS DISEASES OF CHILDREN. 

I can subscribe thoroughly to this recommendation, as it has 
given me admirable results in the treatment of epileptic 
attacks in children. If the tendency to attacks has been 
overcome, the drug should be continued for a period of at 
least one year after the last attack. The dose may be di- 
minished gradually, but it is wise to keep the patient in a 
state of mild brominism. The good effect of the drug can be 
maintained by giving, in conjunction with the bromide, five 
to ten grains of chloral hydrate, a few minims of the tincture 
of digitalis, or of the tincture of belladonna. Bromipin in 
daily doses of ten to twenty-five grammes has been useful. 
Innumerable other drugs have been suggested ; among 
these I will mention cannabis indica, which is particularly 
effective in cases of epilepsy with chronic headaches. The 
oxide and lactate of zinc have been freely recommended, 
but I have seen no beneficial results. The administration 
of iron and arsenic is thoroughly rational ; no doubt the 
restorative effect of these substances upon the blood is the 
indirect cause of improvement in the epileptic condition. 
Some years ago Gowers advised the use of fifteen to thirty 
grains of borax several times daily. A fair trial has been 
given this drug in my clinic and in private practice, but I 
cannot claim more for it than for dozens of other methods.* 
Collins has pleaded in favor of nitroglycerin (in cases with 
a vaso-motor aura), but condemns simulo, osmic acid and 
hydrastis. Flechsig has warmly urged a combination of 
opium and bromides. Opium is given for six weeks ; begin- 
ning with one-half to one grain ; the dose is increased gradu- 
ally until the patient takes eight, ten, or fifteen grains daily ; 
then it is stopped suddenly, and the bromides in thirty- 
grain doses, four times daily, are substituted ; after some 
time the dose is diminished to five or ten grains per day. I 
was ready to adopt this suggestion, as I had for many years 
been using codeine together with the bromide salts. In 
children the doses must be somewhat altered, but the plan 
of treatment, according to my own experience, deserves 
further trial. f 

* Toulouse and Richet have advised the withdrawal of chlorides. Non-salted foods 
are to be given. The bromides will be more effective if less salt is taken into the 
system. 

t Bechterew has advised a combination of bromides and Adonis vernalis. 



EPILEPSY. 83 

The treatment of epilepsy is by no means exhausted by 
the recital of a few medicinal agents. Success depends 
upon other factors. The greatest possible attention should 
be paid to the details of the patient's daily life ; regularity 
in the hours of sleep, in the hours of meals, and careful 
dieting, are essential to proper treatment.* I place my epi- 
leptic patients upon a general mixed diet, including small 
quantities of albuminoids and a liberal vegetable diet, but 
I forbid all indigestible articles of diet. I also limit the 
amount of cereals, and try to check intestinal fermentation 
as far as possible, being moved to this by the result of Her- 
ter's researches. Pastry and sweets of all sorts are strictly 
prohibited. Fresh air at all times, and particularly at night, 
should be insisted upon. Freedom from all emotional ex- 
citement is another recommendation that should not be 
overlooked. Marked ocular defects and other peripheral 
conditions which may cause an epileptic attack are to be 
treated. 

Older patients who have distinct warning of their epi- 
leptic attacks should be provided either with the pearls of 
nitrite of amyl, which they can break up and inhale at short 
notice, or else they can be given a mixture of equal parts of 
chloroform and nitrite of amyl, which can be carried in a 
small phial and can be poured upon a handkerchief for 
purposes of inhalation as soon as the attack is signalled. 
In the case of partial epileptic attacks of definite onset a 
ligature applied around the part first convulsed, to be tight- 
ened quickly as soon as the warning comes, has been rec- 
ommended by Hughlings Jackson, and is serviceable in 
some cases. 

Surgical Treatment. — The surgical treatment of epi- 
lepsy has attracted great attention during the past fifteen 
years or more, ever since it has been known that the motor 
centres in the cortex can be safely and easily reached 
by the surgeon's knife. Surgical interference should be 
considered only in cases of partial epilepsy pointing to a 
definite focus of disease as the starting-point of the entire 
trouble. Nothing seems easier than to expose the centre 

* In recent years rectal olive-oil injections, given at frequent intervals, have proved 
to be of great service. 



84 THE NERVOUS DISEASES OF CHILDREN. 

of the part first convulsed in an epileptic attack, to remove 
this centre, and thus to remove the seat of disease, but, un- 
fortunately, our ardent hopes in this matter have not been 
realized. 

Some years ago Dr. Gerster and myself reported the 
results of operation in ten cases of epilepsy. The cases 
were carefully selected, and if surgical operation could 
have been of avail in any case of epilepsy, it would have 
been so in those we selected for operation ; we were bound 
to admit that in our experience some slight improve- 
ment followed, but also that not a single absolute cure 
had been effected. Since that article was written my ex- 
perience has been fully doubled, and the conclusions reached 
are practically the same. An analysis of the cases reported 
by Starr, which included my own cases, does not justify one 
in taking a more hopeful view of this question. I have 
referred in former writings to the reason for this disap- 
pointment. After an initial injury to the brain, months, 
and sometimes years, elapse before the epileptic habit is 
established. In about the same length of time a general 
cerebral sclerosis has been developed in connection with 
the original focus of disease. We may remove the orig- 
inal focus, but the general sclerosis remains and will act 
as a constant irritant upon the remaining epileptic centres. 

On the other hand, it would be hasty to conclude that the 
surgeon's skill can be dispensed with in these cases.* The 
aim should be to watch carefully for the development of 
epilepsy, and to remove injured tissue at the earliest pos- 
sible date. More than this, I consider it important not to 
await the actual development of epilepsy ; and if the brain 
has sustained any considerable injury to remove the in- 
jured tissues, which, if allowed to remain, constitute a per- 
manent menace to the future health of the patient. We 
shall be able to prevent the development of epilepsy very 
much more readily than we can cure it if once established. 

The surgical procedures in vogue at the present day for 
the cure of epilepsy are trephining and excision of diseased 
tissues. Trephining has been practised for centuries. It 

* The author wishes to direct particular attention to the excellent work done by 
Kocher. 



EPILEPSY, 85 

is a method that entails no special danger upon the life of 
the patient operated upon, and can therefore be tried with 
perfect impunity except in very young- children. White, 
of Philadelphia, and others believe that its only effect is the 
same as that of any operative procedure ; but I am inclined 
to think that if it has any good effect it is in the way of 
relieving increased pressure, and that increased pressure is 
more frequently a real factor in epilepsy than is generally 
supposed, on account of the frequency of cystic formations 
and of the increased ventricular fluid in a very large pro- 
portion of the cases. Excision of tissue would seem to be 
a rational procedure in the earlier stages of epilepsy de- 
veloped after skull injury, or in connection with early cere- 
bral disease ; but the paralysis which so easily results from 
the excision of the motor centres militates somewhat against 
the advisability of this practice, although according to 
Dana's and my own experience such paralysis disappears 
after a few weeks. In cases in which the epileptic attack 
is preceded by sensory or psychic phenomena, a removal 
of the auditory or visual centres, for example, would be a 
serious matter indeed, if not entirely unjustifiable. Many 
a patient would prefer the occasional repetition of an epi- 
leptic attack to a permanent loss of hearing or a permanent 
impairment of sight. 

The only other* surgical procedure which has been 
suggested for the cure of epilepsy is the ligature of one or 
both vertebrals, as was first done by Dr. Alexander, of Liv- 
erpool. The dangers of this operation are so great, and 
the results so uncertain, that it scarcely merits serious con- 
sideration. 

* I do not consider it necessary to refer to the surgical treatment of the eye-muscles 
for the cure of epilepsy except utterly to condemn the practice. As I am treating of 
epilepsy in children, I need not discuss oophorectomy and similar procedures. 



CHAPTER IV. 

HYSTERIA. 

True hysteria is a relatively rare condition in adults* 
This may seem a very heterodox statement to those who 
have been ready to apply the term hysteria to many affec- 
tions of the nervous system which could not be attributed 
to organic lesions. Those who are advised of the recent 
conception of hysteria will not hesitate to indorse the au- 
thor's opinion. The determination of the chief symptoms 
of true hysteria, and the separation of this disease from 
many other functional disturbances which were once classed 
with it, have constituted a signal achievement in modern 
neurology. We have at last succeeded in establishing a 
number of symptoms, chiefly of a sensory character, which, 
when present, enable one to make the diagnosis of hysteria 
with a great degree of certainty, and which do away with 
the necessity of reaching this diagnosis by the process of 
exclusion. As the physician's knowledge of nervous dis- 
eases increases, the diagnosis of hysteria is made more and 
more infrequently. In the author's very large clinical ser- 
vice at the New York Polyclinic the diagnosis of hysteria 
was one of the rarest of all diagnoses made. In other insti- 
tutions with which the writer is connected, particularly in 
the Montefiore Home for Chronic Invalids, hysteria is found 
to be much more frequent, especially among the foreign- 
born inmates. If hysteria is a rare disease in the adult, it is 
still rarer in the child, but when it does occur it is an im- 
portant factor in the future life of the child. Jolly, Ferrier, 
Oppenheim, and others report cases of hysteria occurring 
as early as the second or third year of life. 

* We see much less of true hysteria in this country than in Europe ; it is also far less 
common in England and in Germany than in France and Russia. Broadly speaking, 
the Anglo-Saxon race is less prone to the development of hysteria than the other races 
represented in our population. 






HYSTERIA. 87 

In the development of hysteria, hereditary influences 
are the most potent factors. Charcot and his followers rule 
out all other causes. Given an hereditary predisposition, 
mental overexertion, fright, emotional excitement, exhaust- 
ing- diseases, trauma, masturbation, early sexual irritability 
(Freud) may be the contributing causes. 

English and American authors have had very little to 
say upon the subject. In the large hand-book of Gerhardt, 
Jolly has treated the subject in a very satisfactory manner, 
and in American Cyclopaedias and Systems of Medicine 
the subject has received careful attention at the hands of 
Mills and Lloyd. Henoch has written a very full chapter 
on the subject, but includes under this heading many forms 
of disease which are more properly designated by other 
names. The French authors have naturally had much to 
say on the subject, and they include so many different forms 
under this heading that one is puzzled to know whether 
there is any form of nervous or mental disease that is not 
in some way related to hysteria or influenced by it. 

The symptoms of the disease are truly protean in char- 
acter. No one person, fortunately, ever exhibits even a 
majority of them. Hysteria in one person is very different 
from the disease as it is exhibited in others. It is difficult, 
therefore, to give any one clinical picture of the entire dis- 
ease. It will be best to take up the chief symptoms as they 
occur independently of other diseases, or as they are known 
to complicate other functional and organic diseases of the 
nervous system. Hysteria is characterized by symptoms 
which point to a defect in the various systems and organs 
of the body. Thus we have : 

1. Psychic manifestations. 

2. Motor manifestations. 

3. Sensory manifestations and vasomotor disturbances. 
1. Psychic or Mental Hysteria.— Properly speaking, 

every case of hysteria would come under this heading. 
For, if there is any one feature that distinguishes hysteria 
from other diseases, it is the defect of will-power and the 
excess of the emotional faculties. " I can't " is the pet 
phrase of all hysterical subjects, or still better, ' ; I will not." 
It is not so much a direct lack of power to exert the will, 



55 THE NERVOUS DISEASES OF CHILDREN. 

as a tendency to exert it in perverse fashion. This peculiar 
mental condition is easily recognized, and often leads to the 
diagnosis of hysteria in persons who have other symptoms 
pointing to a much more serious disease. But aside from 
this general hysterical state of mind there are other mental 
conditions which are very typical of hysteria. The most 
pronounced psychic form of hysteria, as observed in chil- 
dren, is that known as hysterical mania. Under great men- 
tal strain or excitement a child is seized with a crying or 
laughing spell, after which it passes into a state of nervous 
excitement in which, as in a little girl, aged eleven, under my 
observation, the child becomes violent, attempts to strike 
others, to injure herself, and to tear her clothes from the 
body, and to do all possible injury irrespective of conse- 
quences. 

Another condition, sometimes classed under the heading 
of hysteria, is observed in young girls, particularly at the 
age of puberty, and often ends in a condition of marked 
acute mania. 1 have seen a number of such cases in girls 
who were over-ambitious and eager to pass their school ex- 
aminations. They would keep up under the excitement of 
the examination, but immediately thereafter, whether suc- 
cessful or not, would become irritable, excitable, sleepless, 
would have laughing and crying spells by turns, would re- 
fuse to take nourishment, and eventually would either re- 
cover under proper treatment, or else pass into a condition 
of typical acute mania with absolute loss of reason, with in- 
tense excitement, and confused delirium. There would be 
no reason to consider these cases in this connection if they 
did not occur in children who have shown either a marked 
nervous predisposition, or who have exhibited hysterical 
symptoms of one kind or another at previous periods. 
Such children, if subjected to severe strain or severe emo- 
tional excitement, are very apt to pass into a condition of 
hysterical mania. 

Other mental conditions which cannot well be separated 
from hysteria, are those known as hystero-epilepsy, cata- 
lepsy, hysterical trance, and the like. Of these, hystero- 
epilepsy is by far the most important, and the gravest dis- 
order. Mills is inclined to consider this form of disease 






HYSTERIA. 89 

very rare among children. In this he is undoubtedly correct ; 
but it has been my good fortune to see a number of classical 
instances of this special form in children, and the account 
I give is based entirely upon personal impressions. The 
gravity of hystero-epilepsy is increased by the fact that 
the children so afflicted are found most frequently in de- 
generate families. Insanity, epilepsy, chorea, chronic alco- 
holism, are the predisposing conditions in the ancestors of 
those who suffer from hystero-epilepsy. In one family I 
have during a period of ten years treated the mother for 
severe hysteria, a daughter at the age of nine for hysteri- 
cal convulsions, and another son and daughter for typical 
hystero-epilepsy. 

On account of this close relationship to true epilepsy, 
cases of hystero-epilepsy cannot be regarded with the in- 
difference which many physicians still display toward hys- 
terical subjects. In many cases it is difficult to decide 
whether the attacks as they occur are more hysterical or 
more epileptic ; and of the attacks occurring in one individ- 
ual some may be typically hysterical, while others may be 
typically epileptic ; and there is some danger in every case 
of severe hystero-epilepsy that with the progress of years 
the hysterical symptoms may vanish and true epileptic at- 
tacks may occur. It is of the utmost importance, before 
passing judgment upon any case, to determine whether the 
attacks are epileptic or hystero-epileptic. 

2. Motor Manifestations. — Every variety of clonic 
and tonic movements occur in hysterical subjects. Many 
of these will be understood best by referring in detail to a 
few cases of typical hystero-epilepsy. 

Some seven years ago I was called to see a lad, then 
thirteen years old, who had been seized with violent con- 
vulsions during the night. I had been informed that these 
convulsions were preceded by great emotional excitement, 
caused by a severe upbraiding which the boy received for 
his misbehavior. After this little domestic scene was over 
the boy went to bed, and was seized with the first attack. 
During the attack he gave a shrill cry ; then began to bark 
like a dog, snapping at everyone who approached him, and 
would pass through the most severe contortions, touching 



go THE NERVOUS DISEASES OF CHILDREN. 

the bed at times only with the head and heels, the back be- 
ing deeply arched as in the famous drawings of Richer 
representing this condition in women. After this the most 
violent jactations of the entire body occurred. During 
these convulsive movements he would snap, bark, and bite, 
then he would suddenly give a leap to the foot of the bed — 
almost tumbling out of bed — yet he always saved himself in 
time. This whole performance would last about two or two 
and a half minutes, then thorough relaxation of all the mus- 
cles would take place, he would fall back exhausted, and 
would then regain consciousness. During the attack there 
was no evidence of consciousness, at least no impression 
could be made upon him through any of the special senses. 
The patient often had as many as two hundred such attacks 
in the course of twenty-four hours ; he was sleepless, refused 
food, and became very much emaciated. After careful nurs- 
ing for a period of six weeks the attacks gradually lessened, 
and eventually he recovered entirely ; but he has shown since 
a deficiency in his moral and mental make-up, and although 
he has been cured of the hystero-epilepsy, it was found 
necessary to place him in a reformatory. This last fact is 
of some interest as showing the relation which hystero- 
epilepsy bears to degeneration of all the mental and moral 
faculties. 

Another case was that of a young girl, aged fourteen, 
who was much worried over the fear that she and the family 
would starve, as the father had daily drummed into the 
child's ears the necessity of economy and the difficulties of 
supporting a family in hard times. The young girl, the 
daughter of a very hysterical and emotional mother, took 
the warning to heart, and for the first time in her life de- 
veloped typical hystero-epileptic attacks. Without warning 
she would fall from a chair, from a sofa, or even on the 
street would pass suddenly into a condition of apparent un- 
consciousness. Wherever she lay she would pass through 
all sorts of contortions, would shriek, now and then would 
give agonizing yells, and would finally end up by a pro- 
nounced crying spell. At other times she would become 
violent, tearing her own clothes, the bed-linen, and every- 
thing that was within her reach in the room. These spells 



HYSTERIA, 



91 



S> 



I 

in 





% 

f 




9 2 



THE NERVOUS DISEASES OF CHILDREN. 



were repeated very frequently during a period of three 
weeks, and then gradually subsided. During all these at- 
tacks the movements seemed to be more or less purposive, 
and yet there was good reason to think, and the girl later 
on confessed, that she was absolutely unconscious of what 
she was doing at the time. These two cases will suffice as 
a general indication of the common form of hystero-epilep- 
tic attacks. 

The distinction between the attacks of epilepsy and of 
hystero-epilepsy is brought out in the following table : 



Epilepsy. 

Aura frequent. 

No distinct cause for each attack. 

Onset sudden. 

Initial cry. 

Eyes open or closed ; pupils often 
dilated widely ; do not react ; roll- 
ing of eyes upward and inward. 

Tonic and clonic convulsions. 



Involuntary micturition or defeca- 
tion. 

Duration of attack only a few min- 
utes. 



The patient often injures himself. 

Biting of the tongue. 
After cessation of clonic movements, 
stupor or somnolence. 



Hystero-Epilepsy. 

No aura ; but, 

Some emotional excitement, direct 
cause of attack. 

Onset sudden or gradual. 

Noises of all sorts during attack. 

Eyes turned up ; pupils normal ; 
often ecstatic expression of coun- 
tenance. 

Either tonic rigidity of muscles or 
extravagant movements (some- 
times purposive). 

No impairment of vesical and rectal 
reflexes. 

Attacks last much longer, followed 
by a condition of trance, or else 
patient recovers consciousness as 
soon as convulsive movements 
cease. 

The patient falls softly, without per- 
sonal injury. 

Biting of the tongue rare. 

Patient may pass into condition of 
trance, or may exhibit signs of fa- 
tigue ; recovery often rapid. 



In addition to the typical attacks of hystero-epilepsy 
there are other forms of motor disturbance due to hysteria. 
The hystero-epileptic attack, while it is the gravest, is in fact 
much rarer than the ordinary hysterical convulsion. The 
latter is characterized by a temporary and imperfect loss 
of consciousness ; by irregular, though sometimes violent, 
twitchings of all extremities, sometimes by the repetition 



HYSTERIA. 93 

of one special form of convulsive movement, such as re- 
traction of the head. When these various convulsive move- 
ments are over, the muscles pass into a state of tonic rigid- 
ity, after which rapid recovery takes place. The entire 
episode is wound up by a fit of crying or laughing, or by 
some other expression of an emotional character. Some of 
the patients pass into a condition of melancholy which may 
last for hours, or into a state of exaltation and even of ec- 
stasy. This is as true of the hysterical attacks as they oc- 
cur in children as it is of those that occur in the adult. 
These attacks can be distinguished from the hystero-epilep- 
tic and from epileptic seizures by the incomplete loss of 
consciousness, by the absence of all regular rhythmical 
convulsive movements, and by the fact that there is neither 
biting of the tongue, nor involuntary micturition, nor any 
of those symptoms which are often associated with hystero- 
epileptic and epileptic attacks. Other hysterical attacks 
consist of spasm of the head and neck muscles, the well- 
known spasm of the oesophagus which gives rise to the 
sensation known as globus hystericus, or to difficulties in 
deglutition if the spasm affects the lower part of the gul- 
let. This oesophageal spasm which occurs in hysterical 
and otherwise neurotic children is a symptom which has 
not met with the appreciation which it deserves. If a 
sound is passed into such a gullet it will be seen that there 
is an actual muscular spasm at the point of constriction, 
and that it requires considerable patience, and sometimes a 
little force, to overcome the contraction ; but the age of the 
child and the fact that the constriction easily disappears, to 
return again under the slightest emotional excitement, is 
sufficient to remove any fear of the constriction being due 
to an organic cause. In this same category we may place 
Spasmodic movements of the diaphragm, of which singultus 
is the common manifestation. This special phenomenon is 
much more frequent in later periods of life than during the 
period of childhood ; but 1 have had under observation an 
entire family afflicted with this special form of hysteria 
who upon the slightest provocation would exhibit this 
symptom. On one occasion I examined the mother, in the 
presence of two daughters, respectively twelve and ten 



94 THE NERVOUS DISEASES OF CHILDREN. 

years of age. Under the excitement of the examination 
the mother was seized with severe singultus, and before I 
had completed the examination both the daughters were 
favoring me with a similar exhibition. The scene would 
have been a comical one if it had not brought home to me 
the powerful influence of example and suggestion, if not of 
heredity, in such cases. 

Spasm of the bladder, increased peristaltic action of 
the stomach and bowels resulting in diarrhoea, upon the 
least emotional excitement, fits of crying or laughing, of 
sneezing and of coughing, are the various forms of hyster- 
ical spasm met with in children, as well as in persons of 
more advanced age. If the hysterical seizure, or better 
said, the hysterical spasms, assume a more extravagant 
character, and if it affects a larger number of the groups of 
muscles that generally act in unison with one another, we 
may have that peculiar condition which is known as chorea 
major. In this form the jactations are often severe and ex- 
hausting. The child or young person assumes the most im- 
possible positions, often suggesting the intention of dra- 
matic effect. From this condition it is but a short step to 
a condition of mild tonic contracture, the limbs retaining 
any position which may be given them. This state of cata- 
lepsy is very often witnessed in conjunction with chorea 
major, sometimes independently of it, but it is a mistake 
to assume that catalepsy occurs exclusively in conjunction 
with hysteria. It occurs in connection with various forms 
of mental derangement, and a condition not unlike cata- 
lepsy is met with in some forms of infantile cerebral paraly- 
sis. 

In hysteria the muscles of the body are not only subject 
to convulsive movements but also to absolute paralysis. 
Every part of the body, including the ocular muscles, may 
be the seat of such palsy, but it is most frequently mani- 
fested in the extremities, in the tongue, and in the vocal 
cords (hysterical aphonia). In some instances there is no 
absolute paralysis of any one muscle or group of muscles, 
but certain functions are paralyzed. The best known exam- 
ple of this is the form which French neurologists have termed 
astasia-abasia. A person thus afflicted is neither able to 



HYSTERIA. 95 

stand nor to walk, but is perfectly well able to use all mus- 
cles while lying on the back. Many have claimed that this 
is simply due to a psychic condition dominated by the 
idea that standing or walking is impossible. This is in all 
probability the proper interpretation ; but the point that 
concerns us here is that it rarely, if ever, occurs except in 
those who exhibit other symptoms of hysteria. 

Hysterical aphonia is very frequent in children, and par- 
ticularly during the period of development. The hysteri- 
cal character of the aphonia is easily recognized by the fact 
that (as the laryngoscope reveals) there is no organic cause 
for the loss of voice, and that the single attacks come on 
very suddenly, as a rule, in the wake of some nervous ex- 
citement, and disappear as rapidly as they have appeared. 
I have had young girls come to my consulting-room who 
would not be able to speak above a whisper, and it has al- 
ways been a special pleasure to have them sing the entire 
scale before leaving the room. A strong faradic brush ap- 
plied to the neck over the trachea is the most persuasive 
master in these cases. Hysterical mutism, an absolute loss 
of speech, is generally the result of severe emotional ex- 
citement. In some instances it is associated with delusions, 
and with other symptoms of mental derangement. 

Other forms of hysterical paralysis, ocular palsies, for 
instance, are not easy to diagnosticate, and in order to dis- 
tinguish them from paralysis due to organic causes the 
physician must have all neurological facts and doctrines at 
his command. It is indeed one of the most difficult problems 
in neurology to distinguish hysterical from organic forms 
of paralysis, but this distinction can be made safely enough 
if the following points are kept in mind : Hysterical paraly- 
sis does not as a rule adhere to anatomical distribution, with 
the one exception that an hysterical hemiplegia may be quite 
as complete as any hemiplegia due to an organic disease. 
But I have not yet met with a single case of hysterical hemi- 
plegia in a child that has in any way suggested, even for a 
moment, the possibility of a hemiplegia due to an organic 
lesion in the brain. In hysterical paralysis the paralysis, as 
well as the anaesthesia which is associated with it, is apt to 
be regional ; thus we have a paralysis of the hand, or a pa- 



g6 THE NERVOUS DISEASES OF CHILDREN. 

ralysis of hand and forearm, or paralysis of an entire extrem- 
ity, with an anaesthesia that, as a rule, develops in proportion 
to the paralysis. This association of regional anaesthesia 
and regional paralysis is, to my mind, very characteristic of 
hysteria. Hysterical contractures are not infrequent. 

From cerebral palsy, hysterical paralysis can be distin- 
guished by the fact that it is not accompanied by increased 
reflexes; and by the lack of marked sensory changes in the 
majority of cases of brain paralysis. The deep reflexes may 
be lively in cases of hysterical paralysis, but 1 have never 
seen them markedly exaggerated, nor are they accompanied 
by the spasticity and tonic contractures which are of such 
frequent occurrence in the case of paralysis due to brain 
disease. The presence of ankle clonus was at one time sup- 
posed to prove the non-hysterical character of an affection ; 
this may b& true in the majority of cases, but since I have 
met with the presence of ankle clonus in some hysterical, 
and even in neurasthenic, affections, I cannot attach much 
importance to this one point. The electrical reactions, it 
should be remembered, are normal both in hysterical and 
in cerebral diseases; we cannot therefore utilize them in any 
way in making the differential diagnosis. 

If the question arises whether paralysis of the upper or 
lower extremities (more frequently of the latter) is due to 
spinal disease, or whether it be purely hysterical, the diag- 
nosis should be based upon the absence of vesical and rec- 
tal symptoms in a case of hysterical paralysis, though to be 
sure there are many cases of spinal disease too in which 
these are not affected. Retention of urine may occur in 
cases of hysteria, but it is only of short duration as a rule, 
and is rarely accompanied by the symptoms of cystitis, as is 
the case in a large number of spinal palsies. In many cases 
of spinal and peripheral nerve disease the electrical reac- 
tions are altered, and the reaction of degeneration may be 
present. This is not the case in hysteria ; but these elec- 
trical reactions are normal also in all those cases of spinal 
disease which are due to disease of the lateral columns and 
not of the anterior or central gray matter. 

All these points of differential diagnosis may in some 
cases prove insufficient, and the diagnosis of hysteria or of 



HYSTERIA. 97 

organic disease of the nervous system will depend upon the 
general agreement or disagreement of symptoms. If the 
physician is aware that flaccid paralysis of a single group 
of muscles, or of one or more extremities, is generally asso- 
ciated with changes in the electrical reactions, and with loss 
of reflexes in case these symptoms are due to organic dis- 
ease, and if he remembers, furthermore, that spastic forms 
of paralysis are associated with increased reflexes, with nor- 
mal electrical reactions, and with normal sensations, he will 
have little or no difficulty in arriving at a correct diagnosis. 
Hysterical paralysis is subject, moreover, to greater changes 
than the organic palsies are. While under certain condi- 
tions the very persistence of the symptoms for a long period 
of time, followed by a sudden change, is extremely char- 
acteristic of hysterical paralysis, the diagnosis becomes 
still more certain if the hysterical paralysis is associated 
with other symptoms, such as are known to be purely hys- 
terical. I refer particularly to the presence of rigidity, 
anaesthesia, or typical and complete hemianaesthesia, and to 
the occurrence of major or minor hysterical attacks ; but the 
difficulties are still further increased by a fact to which 
Seguin called particular attention some years ago, that 
hysterical paralysis or hysterical symptoms often compli- 
cate organic disease. 

French authors have reported a number of cases of extreme progressive 
wasting of muscles in hysteria. Hirt has recorded a case in a young girl, so 
astounding in the rapidity and degree of development of the atrophy that it 
almost challenges belief. 

3. Sensory Symptoms. — From the preceding pages it is 
evident that the sensory symptoms often enable one to make 
a positive diagnosis of the hysterical character of the affec- 
tion when other symptoms would leave some room for 
doubt. Every form of sensation may be affected, and it 
may either be exaggerated or dimished. Hysterical 
hyperassthesia and hysterical anaesthesia are of common 
occurrence. If hyperassthesia exist there is an unusual 
sensitiveness to the slightest touch or to the slightest pain- 
ful impression. This hyperassthesia is most prominently 
developed in the region of the ovaries, in the skin over the 
7 



98 THE NERVOUS DISEASES OF CHILDREN. 

spinal column, each spinous process being so sensitive to 
touch that the patient cries out from pain, however light 
the touch may be. The hyperaesthetic areas, if stimulated, 
are very apt to cause distinct hysterical attacks in those 
prone to such seizures, and the hyperaesthetic areas may 
become true hysterogenic zones. These hysterogenic 
zones have been studied with great care by the French 
school, but we cannot enter upon the subject more fully 
here, and leave it with the simple statement that there are 
some hysterogenic zones which are not hyperaesthetic. 

Hysterical anaesthesia is still more frequent than the 
exaggerations of sensory impressions. The anaesthesia 
may not only include every form of ordinary sensory per- 
ception but also the special senses. The distribution of 
this anaesthesia is one of the most valuable signs of hysteria. 
It occurs in the form of a complete hemianaesthesia, or in 
the form of a regional anaesthesia. The hemianaesthesia is 
limited strictly to one-half of the body, but in this half it 
is often absolute, and the completeness of the anaesthesia is 
the very feature which should make us suspicious of its 
hysterical nature. If a girl or a boy, or for that matter any 
person at any age, present absolute anaesthesia to all forms 
of ordinary sensation, and in addition exhibits blindness of 
one side or a typical hemianopsia, deafness, loss of taste, 
and of smell in the same half of the body, such a person 
has undoubtedly hysteria. In this country hysterical 
hemianaesthesia is relatively rare, excepting in the Russian 
and French elements of our population, but in these it can 
be studied easily and satisfactorily. 

If an entire half of the body is not anaesthetic, the re- 
gional character of an anaesthesia is sufficient to lead one to 
the diagnosis of hysteria. By regional anaesthesia I mean 
anaesthesia of a well-marked division of the body, say of 
the hand, of the forearm, of the leg, the hips, or of circum- 
scribed areas in any part of the body without reference to 
the anatomical distribution of the sensory nerves. (Fig. 45.) 
As this regional anaesthesia is frequently associated with a 
regional paralysis the hysterical nature of both becomes 
very evident. The hysterical nature of the anaesthesia is 
not only made evident by its peculiar manner of develop. 



HYSTERIA. 



99 



ment, but also by the manner in which it often disappears. 
In a number of cases in which I have studied this anaesthesia 
carefully it would recede inch by inch, but always main- 
tained a certain level in the arms or legs without any ref- 
erence to the well-known sensory areas. Hysterical pa- 
tients do not present anaesthesia of the anterior or posterior 
surface of a limb, but the anaesthesia generally involves 
both surfaces. The hysterical nature of sensory disturb- 
ances is also established by the powerful effect of hypnot- 




FlG. 45. — The Three Types of Distribution of Anaesthesia in Hysteria: Hemianaes- 
thesia, Segmental, and Disseminated. Z, Hysterogenic Zones. (Dana.) 

ism, and of mere suggestion, which is often sufficient at 
least temporarily to dislodge a complete anaesthesia. The 
anaesthesia may furthermore be transferred from one limb 
to another, not merely by the action of magnets, or by the 
action of metals as was formerly supposed, but, I am con- 
vinced, by any form of suggestion powerful enough to pro- 
duce a strong psychic impression. It is on the theory of 
suggestion that we must explain the miracles ascribed to 
magneto-therapy or metallo-therapy, that played such an 
important role about twenty-five years ago in France. 

Of the special senses, vision is most frequently subject 
to hysterical disorder. In some there is true photophobia, 
in others a diminution of visual perception is more common, 



nro 



00 



THE NERVOUS DISEASES OF CHILDREN. 



and the patient may indeed be unaware of the existence of 
such diminished visual sensation, which is the best proof 
that it is not intentional or simulated. The retina may be 
entirely insensitive to light, there may be distinct limita- 
tion of the visual field, or there may be a complete loss 
of every form of visual perception in one eye. (Fig. 44.) 
Bilateral loss of sight is said to occur in hysterical patients, 
but as a rule it is simply transitory. I have not had op- 
portunity to see this special visual defect in children. 

Visceral hysteria deserves a passing notice ; the paral- 
ysis of the bladder and increased peristaltic action of the 




o. s. 



o. D. 



Fig. 46. — Hysterical Loss pf Color Sense and Limitation of Visual Field. Color 
Sense Absent in Left Eye and Field Contracted ; in Right Eye Field less Con- 
tracted ; Order of Appreciation of Colors from Without In was Yellow, Violet, Blue, 
Red, Green. (After Peck, from Dana.) 



stomach and bowels have been mentioned in connection 
with the motor disturbances of hysteria, but there are two 
other forms of hysterical manifestations that are worthy of 
further mention. The one form is hysterical anorexia. 
Patients in this condition absolutely refuse food, and if they 
take it, vomit it at once. In some instances this is accom- 
plished without effort on the part of the patient and seems 
entirely unintentional. In other instances, again, the pa- 
tient deliberately sets to work to eject the contents of the 
stomach, and does not give up the effort until she has suc- 
ceeded. In my service at the Montefiore Home I frequent 



HYSTERIA. 101 

ly had occasion to witness the antics of an hysterical girl, 
aged fifteen, who immediately upon taking food would go 
into a corner of the room, begin violent gagging movements, 
and would succeed within a period of three or four minutes 
in ejecting the entire contents of the stomach. In this girl, 
who was not possessed of any insane delusion regarding 
the character of the food, the act seemed purely volitional, 
and it was a surprising fact that like many other hysterical 
patients with anorexia, she was not so much reduced in her 
physical condition as a person would have been who had 
persistently starved herself. She evidently obtained food 
and retained it ; but how she got it and when she took it 
it was difficult to determine, since she was watched care- 
fully night and day. She was treated by rather heroic 
measures in the form of forced feeding and hydro-thera- 
peutic measures ; she was allowed to leave the institution 
after she had shown her willingness to take food and re- 
tain it. 

Distention of the stomach and bowels, representing a 
total paralysis of the muscular walls of the stomach and in- 
testines, occurs frequently enough, and more particularly in 
children. The stomach is apt to be distended to an enor- 
mous degree, and the bowels dilate in such a way as to give 
the appearance of the abdomen in a case of large-sized 
tumor. I have in mind the cases of twin brothers who pre- 
sented the following peculiar condition : The one brother, 
at the age of fourteen, was true to the neurotic stock from 
which he descended by suddenly developing a tremendous 
meteorismus, which increased from day to day until the 
abdomen was so distended that the skin seemed ready to 
burst. There was some anorexia and constipation. The 
constipation was occasionally relieved, with the result of 
temporarily diminishing the distention of the bowels, but 
in a very few hours the intestines would resume their for- 
mer state. Every possible measure was resorted to, to re- 
move this distention, but it persisted stubbornly for some 
weeks, when finally, after the introduction of large enemata 
of an infusion of valerian, the distended abdomen suddenly 
collapsed. A few days later the twin brother of this patient 
began the same performance, and went through exactly the 



102 THE NERVOUS DISEASES OF CHILDREN. 

same experiences. The abdomen became distended to almost 
the same degree as in the brother, and after it had lasted 
about the same length of time his abdomen also collapsed in 
very much the same way. These boys not only came from 
very neurotic stock, but had been very much pampered in 
their early training, which fertilized the soil that had been 
prepared for the development of every form of hysteria. 

Diagnosis. — It would be impossible to state the points 
of differential diagnosis between hysteria and other affec- 
tions without repeating everything that has been said in 
the preceding paragraphs. It is worth while stating once 
more that the diagnosis of hysteria should be made only in 
case an organic affection can be positively excluded, and if 
the well-recognized symptoms of hysteria, particularly hys- 
terical seizures and hysterical sensory changes are present. 
Let the student also bear in mind that hysteria and hypo- 
chondriasis are not one and the same disease, though one 
is often mistaken for the other. If a boy is fearful of a dis- 
ease with which he supposes he is afflicted, we have no 
hesitation in saying that he is a hypochondriac ; but let a 
girl exhibit exactly the same symptom, and she is at once 
put down as an hysterical subject. To distinguish between 
the two conditions is not always an easy matter, but in 
hypochondriasis the patient is generally possessed of a few 
notions regarding his own bodily condition, and these make 
such a deep impression upon him that his entire ego is af- 
fected by it. His supposed affliction is constantly before 
his mind, and dominates his entire being. In cases of hys- 
teria there is no such introspection as in hypochondriasis. 
The bodily ailments or bodily peculiarities are far more 
numerous, but they are subject to greater changes, disap- 
pear for a time and then return again, and are not pushed 
to the fore quite as much as in cases of hypochondriasis. 
In the latter, moreover, the depression is, as a rule, greater 
than in hysteria, and in hysteria there are other symptoms 
which prove the presence of an hysterical affection. The 
difficulties of a differential diagnosis are still further in- 
creased by the fact that a mixture of the two conditions 
(hysterical hypochondriasis) is not uncommon, and it must 
be left to the physician to determine, by a close analysis 



HYSTERIA. 103 

of the symptoms, whether there is more hysteria or more 
hypochondriasis in the symptoms which the patient pre- 
sents. 

Pathology. — In discussing the pathology of hysteria 
there is much room for theorizing, but there are very few 
facts to guide us. That it is a functional disease is con- 
ceded on all sides; but there is no other disease in which 
the loss of function may be so absolute as in hysteria. By 
some mechanism or by some influence which we cannot 
yet understand, an entire hemisphere is temporarily invali- 
dated, or else we could not explain the typical and com- 
plete hemianaesthesia so common in hysteria. Meynert 
endeavored to give an anatomical explanation, but failed. 
How this loss of function of one hemisphere is effected, and 
whether it is similar or not to the occurrences that take 
place under hypnotic influence, we are not prepared to say. 
The transfer of sensory disturbances from one-half of the 
body to the other, would go to show that the two halves 
of the brain are evidently in sympathy with one another 
(to put it broadly) in this disease, and that they can be al- 
ternately affected. The highest centres are evidently im- 
paired in hysteria, cortical inhibition is removed, and the 
lower centres have full sway.* It is quite in keeping with 
this view that even the reflexes are frequently exaggerated 
in hysteria as they are in organic disease in which the 
changes in the motor fibres of the pyramidal tract interfere 
with the proper transmission of cortical influences. We 
cannot properly speak of the morbid anatomy of hysteria, 
for the entire conception of the disease would have to be 
altered if we could suppose the existence of such. Some 
post-mortem findings have been reported, but they were 
evidently accidental complications. 

Duration and Course. — Once hysterical always hys- 
terical, would seem to be the general opinion with regard 
to these subjects, but this is not quite accurate. The mani- 
festations of this disease often disappear for years, and it is 

* Breuer and Freud have attempted a psychological explanation of hysterical phe- 
nomena and have ascribed these to a " hypnoid " condition in which there is a division 
of consciousness, and a limitation of the power of association. Freud has directed at- 
tention to the diminution in the strength of concepts in hysterical subjects, and to the 
ready change from psychic to physical innervation. 



104 THE NERVOUS DISEASES OF CHILDREN. 

one of the commonest experiences to record the disappear- 
ance of an hysterical paralysis or of an hysterical anaes- 
thesia. The tendency to relapses is extremely great, and 
symptoms that have disappeared for years may occur un- 
der any emotional excitement. 

Treatment. — The treatment of hysteria must be con- 
sidered with reference to the hysterial predisposition and 
with reference to the special hysterical symptoms. The 
hysterical predisposition is unfortunately either inherited 
from a neurotic ancestry or it has been specially fostered 
by an hysterical environment. In hysteria, as in other men- 
tal affections, I have often doubted whether heredity is the 
marked factor that it is generally supposed to be, or, if we 
grant the hereditary tendency, whether the hysterical man- 
ifestations would be as frequent as they are if a serious at- 
tempt were made to change the surroundings of such chil- 
dren. In the majority of cases the early training has been 
defective, and an example constantly before the child of an 
hysterical mother or other hysterical relatives is sufficient 
to engender the disease in its fullest form. The first and 
most important principle of treatment, therefore, is the ab- 
solute separation of the child from the family. It is a great 
pity that this is so rarely urged by the physician, and still 
more rarely permitted by the parents. Only the more in- 
telligent parents can be made to understand that an utter 
stranger, if properly qualified, may train a child far better 
than its own mother can. At all events there is nothing in 
the treatment of hysterical children as important as plac- 
ing them under the influence of a sober-minded, intelligent 
nurse or teacher who will devote herself or himself to study- 
ing the peculiarities of the child, and who will make a seri- 
ous attempt to foster the good qualities and to counteract 
the vices. If this course that has been suggested is per- 
sisted in, it should be followed not for weeks or months but 
for years, and I am confident that if this is done during the 
formative period of a child's mind and character, an excel- 
lent result will follow in many instances. I base this upon 
my own experience, and have often stated to parents that 
if they will not allow me to pursue such a plan of treatment 
I prefer not to attempt any. 



HYSTERIA. 105 

The Weir-Mitchell treatment, which is so effective in 
many cases of adult listeria, is also of the greatest value in 
the hysteria of children ; but I consider it to be one of its 
special advantages that it presents the first and easiest op- 
portunity for the separation of the child from its immediate 
surroundings. Select a nurse carefully, place the child en- 
tirely in her charge, and many a parent will soon be con- 
vinced of the good that comes from such separation. In 
addition to the moral influences which may be exerted in 
this way the physical health of the child can be looked 
after. Bad habits of diet and of general hygiene can be 
corrected, and a child whose general physical condition 
has been far below par up to the time of its entering upon 
the rest cure, may be turned into a strong and vigorous be- 
ing. Drugs, I am very certain, have but little influence 
over such conditions. The usual hysterical remedies, such 
as asafcetida, valerian, and the like, may be employed, but 
they will accomplish little which cannot be accomplished 
by other means as well. If the effect of a drug depends 
upon its disagreeable taste, suggestions of a different char- 
acter will prove quite as efficient ; and above all the phy- 
sician should avoid the possible risk of engendering the 
idea in a growing child that there is a special drug which 
it may resort to for any annoying or painful sensation. 
There is nothing more disgusting than the habit so freely 
practised by many physicians of giving valerian or asafce- 
tida or morphine to children or adults, whenever they pre- 
sent symptoms which smack of hysteria, or which cannot 
be interpreted otherwise by the attending medical adviser. 

The special symptoms of hysteria call for distinct forms 
of treatment, but whatever these special symptoms may be, I 
always consider it wise to bear the general hysterical condi- 
tions in mind, and to employ, in addition to the special treat- 
ment, those general measures which have an excellent invigo- 
rating effect upon the nervous system. I refer more particu- 
larly to the proper use of hydrotherapeutic measures. The 
treatment of hysterical aphonia was referred to above. Sim- 
ple faradism is all that is needed in most instances, and if this 
is not sufficient, regular vocal exercise in the hands of an in- 
telligent teacher or nurse, will bring about the desired effect. 



106 THE NERVOUS DISEASES OF CHILDREN. 

I have never found it necessary to apply the faradic current 
to the inside of a throat, but of course would not hesitate 
to do so in case the external application did not prove suf- 
ficient. Blistering or the application of the static current acts 
as a powerful form of local suggestion. In hysterical paral- 
ysis of the limbs, the use of a strong faradic current is gener- 
ally the most effectual remedy. The current applied know- 
ingly, not mercilessly, will gradually induce the patient to 
attempt similar contractions ; and if to the use of the faradic 
current be added the encouraging words of the nurse or 
physician, who should not, however, accuse the child of 
simulation, and if other measures, such as massage, be em- 
ployed, the hysterical paralysis will disappear in the course 
of time. But many of the cases are extremely stubborn 
and may require more vigorous measures, or more power- 
ful suggestion, before they yield to treatment. It is of 
some importance at times to prove to the child that it can 
use its limbs very much better than it supposed. Place it 
in the middle of a room quite by itself, make it stand or 
crawl or walk, and if once shown that it can do so, the para- 
lytic symptoms may rapidly disappear. But the treatment 
of hysterical patients of all ages and of all classes demands 
unusual tact and patience on the part of everyone con- 
cerned in the treatment of the case. 

The sensory disturbances of hysteria are best influenced 
by the use of the cold douche or of the faradic current, 
particularly by the employment of the faradic brush. If 
such measures as I have suggested are not sufficient to re- 
move an hysterical paralysis or an hysterical anaesthesia 
the effect of suggestion, hypnotic or otherwise, may be 
attempted. 

The visceral disturbances common in hysteria also de- 
mand special treatment. Some of the measures to be em- 
ployed were referred to in connection with the hysterical 
twins mentioned above. In cases of hysterical anorexia 
patient efforts should be made to induce the patient to take 
small quantities of food, and if these are not retained forced 
feeding must be resorted to ; but never, if you can avoid it, 
resort to rectal feeding, for the patient who has discovered 
that she can be fed in that way will continue to refuse food 



HYSTERIA. I07 

very much longer than she would otherwise. Lastly, the 
treatment of hysterical attacks may be managed in very 
much the same way as other hysterical manifestations are 
treated. First of all, the patient should receive the general 
anti-hysterical treatment, and should be given some drug 
which will act as a powerful irritant or a powerful form of 
suggestion when the attack is imminent. I have found 
nothing better than a sudden douche of cold water, or cold 
flagellations on the head and chest, or the inhalation of ni- 
trite of amyl. The latter has this to recommend it, that it 
may be used with all the more assurance in those cases in 
which the suspicion of epilepsy cannot be altogether ex- 
cluded. Lavender and ammonia are also efficient in some 
cases in which the hysterical attack can be inhibited by any 
such simple measure. If an hysterical attack continue for 
a prolonged period of time it may be brought to an end 
by the brisk use of cold douches, by the application of a 
strong faradic current, or by pressure over the ovaries, 
after the age of puberty, if these organs be oversensitive. 



CHAPTER V. 

CHOREA. 

Among the neuroses of childhood none is worthier of 
careful study than chorea. The name is made by some to 
include a number of varying conditions, but its use should 
be restricted to designate a functional disease characterized 
by irregular, involuntary twitchings of some or all of the 
muscles of the body. These movements cease, as a rule, 
during sleep. 

Synonyms. — This neurosis is also known as St. Vitus's Dance and the 
Chorea of Sydenham. Both these names deserve to be used, since the former 
implies its historical origin, and in the latter is preserved the name of the 
famous English physician who first described its most characteristic symp- . 
toms. Scelotyrbe and Melancholia saltans, are terms occasionally used, 
though almost obsolete. In German text-books the disease is called Chorea 
Minor, in contradistinction to Chorea Major, a neurosis of a purely hysterical 
character.* 

Etiology. — Chorea is distinctly a neurosis of childhood 
and early adolescence. The vast majority of cases begin in 
very early youth, though Sinkler, many years ago, reported 
two cases in persons over eighty years of age. Careful sta- 
tistics have been gathered with reference to this disease, 
the most elaborate being those reported by Dr. Stephen 
Mackenzie, in 1887, f° r tne British Medical Association Col- 
lective Investigation Committee. Of four hundred and 
thirty-nine cases reported by this committee thirty-four per 
cent, occurred between the ages of five and ten years, forty- 

*The name chorea, Greek x°P el ' a , can be traced back to the dancing mania of the 
middle ages. During a severe outbreak of this psychic disturbance in Strasburg, in 
the early part of the fifteenth century, the chief magistrate of that city ordered those 
affected with this dancing mania to repair to the chapel of St. Vitus, in Zabern, a small 
village not far from Strasburg. The name, St. Vitus's Dance, is the only point of affin- 
ity between the dancing mania of old and the typical chorea of the present day. 



CHOREA. IO9 

three per cent, between the ages of ten and fifteen years, 
and sixteen per cent, between the ages of fifteen and twenty 
years. The largest number of attacks were found to occur 
in the thirteenth year of life. Some cases, however, oc- 
curred very much earlier than this average percentage 
would indicate. I have seen several cases of genuine chorea 
in children under one year of age, and many more in 
children up to the age of three years. That chorea is 
also occasionally congenital must be admitted : Sinkler re- 
fers to a case of this description due to fright of the mother 
during pregnancy. That there is hereditary predisposition 
to chorea is also evident, for the disease is developed more 
readily in children of choreic mothers and also in those 
whose parents are afflicted with epilepsy or migraine. 

All authors are agreed as to the greater liability of the 
female sex. Within a period of three years I had seen 184 
cases of chorea, in which 136 were females and 48 males. 
Sinkler among 328 cases reports 232 females and 96 males. 
Gowers, who has tabulated the largest number of cases, finds 
only 365 boys among 1,000 cases. 

Dr. Weir Mitchell has studied the relation of races to 
the development of chorea, and claims that the negro race 
is almost exempt. I have no means of saying whether this 
is approximately true, but from my own experience I can 
assert that it is by no means rare among that race, but that 
among them it is if anything more frequent in boys than 
in girls. The disease is very common in Hebrews, as are 
many other neuroses. 

Climate seems to exercise but very little influence upon 
the development of the disease proper, or upon the causa- 
tion of the individual attack ; it occurs quite as freely in 
cold countries as in warm, in northern as in southern lati- 
tudes, but there is an undoubted seasonal influence. The 
great majority of attacks occur in the spring. Drs. Mitch- 
ell and Lewis have made an elaborate research on this point, 
and claim that it is not so much variation in temperature 
or in humidity that causes the attacks, but that there is a 
decided correspondence between the number of attacks of 
chorea and the number of rainy and cloud)' days ; and then 
again between the attacks of chorea and the number of storm- 



IIO THE NERVOUS DISEASES OF CHILDREN. 

centres that pass over Philadelphia. Too much reliance 
should not be placed upon these statements until confirmed 
by similar researches in other cities. Putnam could not 
trace the same influences in Boston, and Gowers cannot 
accept the conclusions of Lewis for the conditions under 
which chorea occurs in England. These factors, if power- 
ful at all, are of more importance in bringing about a recur- 
rence of attacks that in giving the first impetus to the dis- 
ease. 

The causes which lead most directly to the development 
of St. Vitus's Dance are (in the order of their importance) 
fright, various acute diseases, such as articular rheuma- 
tism, scarlatina, and cardiac disease which so often ac- 
companies the acute affections just mentioned. Gowers is 
inclined to regard fright as " the only immediate cause that 
can be traced with any frequency." It was the direct cause 
of chorea in 56 of my 184 cases. As a rule, the first symp- 
toms of chorea appear within a few days of the fright ; in 
some cases within a few hours, and even within the first 
hour. I had under observation for a long time a child that 
lived near the Brooklyn Theatre at the time it was destroyed 
by fire ; the child was startled by the sight of the flames, 
and within a few hours began to twitch, and soon developed 
a severe attack of chorea which lasted for months. In one- 
case the sight of a street brawl, in another the sight of a dead 
body, was directly the cause of the choreic attack. In chil- 
dren who have once had chorea very trivial occurrences 
are apt to bring about a recurrence. I have known a slight 
overstrain at. school, the unexpected report of a pistol, a 
severe thunderstorm, or a scolding by a parent to be suf- 
ficient to bring on an attack. 

The relation of acute rheumatism to the development of 
chorea has always been a matter of dispute. Some claim 
an absolute causal relation between the two, others insist 
that it is nothing more than a coincidence if one disease 
follows upon the other. Statistics upon this point are not so 
satisfactory as they might be, for cases have been reported 
in which it was stated that acute rheumatism has occurred, 
but it is not clearly made out that the rheumatism actually 
preceded the onset of the chorea. See reported the occur- 



CHOREA. Ill 

rence of acute rheumatism in about one-fourth of his cases, 
and in these figures he is supported by the conclusions of the 
British Medical Investigation Committee and by Gowers.* 
It is a curious fact the rheumatism does not seem to pre- 
cede chorea nearly so frequently in earlier years as it does 
in those cases which occur between the ages of ten and fif- 
teen years. From this we must infer that accidental coin- 
cidence plays a very much greater role than many are will- 
ing to concede. This must unquestionably be the case with 
the statistics furnished by Dr. Hamilton, who found twenty 
per cent, of all school children choreic or affected with some 
similar disorder. I could find a satisfactory history of the 
chorea coming on after rheumatism in only 20 of 184 cases.f 
Hirt, in his recent text-book on Nervous Diseases, thinks that 
there is a common toxic agent which, if it affects the cor- 
tex, will produce choreic movements; if it affects the joints 
chiefly, will give rise to acute rheumatism. This may be a 
rather hasty conclusion, but there is enough truth in it to 
say that the development of chorea is more probable in per- 
sons who have had rheumatism than in those who have not. 
The majority of recent writers, including Herringham, 
Mackenzie, Oppenheim, Gowers, Osier, and others insist 
that an intimate relation exists between chorea and rheuma- 
tism ; but the question arises whether heart disease, which is 
so frequently associated with rheumatism, may not be in part 
responsible for the development of chorea. Statistics, in 
order to be satisfactory, should be collected very much 
more carefully than they have been hitherto, in order to 
prove in how few, or in how many, cases of chorea the first 
attack has been preceded by rheumatism or cardiac disease. 
We can readily understand that fright should be a more 
powerful agent for evil in cases in which cardiac disease 
has preceded the existence of chorea. 1 have found satis- 
factory evidence % of cardiac disease preceding the devel- 
opment of chorea in only 20 of 184 cases. The bearing that 

* Starr has tabulated 2,476 cases (by various authors); in 662 (twenty-six per cent.) 
there was a history of preceding rheumatism ; in 502 there was cardiac disease. 

t Osier states that there was a history of rheumatism in 15.8 per cent, of cases which 
he had examined carefully. 

% The mere existence of a murmur has been taken by many as evidence of cardiac 
disease ; in the fewest reports is there any accurate diagnosis of the cardiac condition. 



112 THE NERVOUS DISEASES OF CHILDREN. 

heart disease may have upon the pathology of chorea we 
shall have occasion to refer to later on. • 

There is a marked tendency at the present time to 
overrate the infectious origin of chorea. One may claim 
that it is at times a post infectious disorder. To claim 
more would be unwarranted. 

There has been much talk about reflex chorea, as about 
the reflex origin of many other neuroses, but he who sees 
with only half an eye will soon convince himself that these 
reflex theories are but a poor makeshift. Of all the cases 
of chorea that I have seen, I have found but very few that I 
could consider due to any peripheral exciting cause. I have 
convinced myself that in a few cases the presence of intes- 
tinal parasites was the cause of a transitory chorea, which 
disappeared as soon as the parasites were removed, but I 
am not convinced that nasal or ocular trouble, of which so 
much has been made of late, ever leads to true chorea. If 
these troubles prove an inconvenience to the child, some 
choreiform habits may for a time be established, but in 
such cases the cardinal symptoms of St. Vitus's Dance are 
wanting. 

There is a curious relation between epilepsy and chorea. 
Gowers refers to epilepsy developing from chorea, and I 
have seen a case in a woman of forty, and another in a child 
of ten years, in which severe chorea set in after the cessa- 
tion of epileptic attacks. 

Symptoms. — Involuntary and irregular movements of 
any muscle or group of muscles of the body constitute the 
chief symptom of chorea. The muscles of the hands and 
fingers, and of the face and tongue are most often affected, 
but the leg and trunk muscles are at times involved. These 
movements are aggravated by volitional effort either of the 
muscles affected or of some other group of muscles. Thus 
the choreic movements of the hands will often become very 
much more intense if the child is told to stand absolutely 
still, or if while one hand is being examined it is asked to 
grasp something with the other hand. If the patient at- 
tempt to keep the affected part absolutely quiet he may 
succeed in doing so for a few seconds, but after that the 
movements will become more intense. 



CHOREA. 113 

The choreic movements may affect only one extremity ; 
they may involve one-half of the body (hemichorea), or they 
may be generalized. In 184 cases, 35 were cases of right 
hemichorea; 32 of left hemichorea; and in 117 cases the 
choreic movements were general. 

As a rule the choreic movements are so evident that no 
special examination is needed. As the child enters the con- 
sulting-room the most superficial inspection is sufficient for 
a diagnosis; but in other cases, particularly during the early 
stages of the disease, the choreic movements are discovered 
only upon careful examination. If there is any doubt what- 
ever about the condition, I ask the child to place its hand 
quietly upon my own, or between my two hands ; the irreg- 
ular choreic movements will, if present, be easily seen or 
felt. The true nature of the trouble, which may have ap- 
peared to be nothing more than "a slight nervousness," may 
thus be detected. If any further corroboration is needed, an 
examination of the tongue, as a rule, reveals the true char- 
acter of the disease, for there are very few cases of chorea 
in which if the movements of the extremities are ever so 
slight, the tongue does not exhibit very marked choreic 
twitching. These tongue movements are slow, coarse, 
sometimes rhythmical. In advanced cases, if the tongue 
is protruded the mouth is opened much more widely than 
necessary, the eyelids and eyebrows are raised in the same 
effort, and then through a choreic movement of the mas- 
seters the tongue may be caught between the teeth. These 
movements combined give rise to what, in a former article, 
I called the " facies " of chorea. 

The movements of the choreic patient are not only 
irregular but are often awkward to the extreme. This is 
clearly shown in the attempt to open or button the cloth- 
ing, in raising a glass of water to the lips, or in attempting 
to hold the pen in writing. This awkwardness often in- 
duces great irritability on the part of the child ; but how- 
ever annoying the movements may be, it is only in a very 
small proportion of the cases that they lead to a condition 
of exhaustion. A few years ago I had occasion to observe 
a little girl, six years of age, the child of healthy and intel- 
ligent parents. It had passed successfully through one 



114 ' THE NERVOUS DISEASES OF CHILDREN. 

attack of chorea , and in the second attack, coming on after 
a fright, the movements were extreme, and sleep was so 
poor that within a few weeks the child died from exhaus- 
tion. A weakened but not diseased heart unquestionably 
assisted in bringing about this early fatal termination. 
It is a fortunate circumstance that in almost all these cases 
the movements cease during sleep, and that the child is 
thus able to recover partially from the exhaustion caused 
by the movements during the day. 

Some weakness of the muscles is frequently associated 
with choreic movements. The term paralytic chorea has 
been proposed for those cases in which there is marked 
paralysis, but as there is more or less weakness in the 
majority of the cases, and often more awkwardness than 
weakness, there does not seem to me to be sufficient excuse 
for the introduction of this term. 

Speech is frequently involved. This is in the nature of 
a dysarthria rather than an aphasia, the choreic movements 
of the tongue and laryngeal muscles making speech dif- 
ficult and often unintelligible. In some cases there is a 
little awkwardness of articulation, in others hasty articu- 
lation leading to the repetition of words, and in some a 
peculiar condition of speech which is in part due to diffi- 
culties of articulation, and in part to choreic movements 
of the respiratory muscles necessitating rapid breathing. 
Deglutition may be difficult, the tongue is frequently bitten, 
and from the awkwardness in the use of the knife and fork, 
and in passing food to the mouth, the little patient is much 
annoyed and is an ungainly sight for others while at his 
meals. 

Laryngeal chorea, pure and simple, occurs somewhat 
rarely, and consists of choreiform movements of the muscles 
controlling the vocal cord. The result is a peculiar ex- 
piratory noise like a bark, which is repeated at short in- 
tervals. These cases are often mistaken for cases of hys- 
terical bark ; but the general restlessness, the age of the 
patient, the choreic movements of the tongue and fingers, 
should leave little doubt regarding the diagnosis. I re- 
member a little girl, aged ten, who began to bark after a 
sudden fright ; her case had been diagnosticated as hysteria 



CHOREA. 



115 



by several eminent physicians, but there was no element of 
true hysteria in the case. She recovered promptly under 
the usual rest treatment. There is little doubt in my own 
mind that this represents the rarest form of chorea. 

The electrical reactions are sometimes slightly altered 
in cases of chorea. Rosenthal, Benedict, and others have 
found an increased response to the faradic and galvanic 
currents on the part of the muscles and nerves of the af- 
fected side. Some have even asserted that the reaction of 
degeneration with qualitative galvanic changes occurs in 
some instances, but I am inclined to doubt the correctness 
of this statement. I have never found a similar condition 
although I have frequently examined patients with this end 
in view. If such electrical changes were present I should 
suspect the presence of multiple neuritis, as this has been 
known to occur together with chorea ; just as I might sus- 
pect this same complication in cases of marked sensory dis- 
turbances, for the rule is that in uncomplicated chorea sen- 
sation remains undisturbed. 

Mental disturbance has been frequently referred to by 
many writers as a complication of chorea. It is surely 
not a very frequent occurrence, except that in the cases 
of chronic chorea (probably a different disease) the ten- 
dency to dementia is very marked. The impression I 
recorded a few years ago seems to me to represent the 
truth of the matter : " The mental calibre of children who 
develop chorea is rather above than below par. Children 
who by means of a better mental development stand head 
of the class, who work for prizes and earn them, children 
who are under constant mental strain, and about whom 
parents and teachers make much ado, are the ones most apt 
to be attacked by chorea." In some instances a violent 
mania is developed early {chorea insaniens), but it is much 
rarer to find this sequence of events than to observe cases 
of acute mania, particularly among young girls, in whom 
the movements of the extremities and of the tongue are 
typically choreic. Irritability of temper is perhaps the 
most frequent mental condition associated with chorea ; but 
this is natural enough if we consider the very annoying 
movements and the difficulty the child sometimes expe- 



Il6 THE NERVOUS DISEASES OF CHILDREN. 

riences in making itself understood. At times, instead of a 
condition of mania, a condition of apathy and depression is 
present in patients afflicted with chorea ; but I am inclined 
to think that this is only true of patients who inherit a pre- 
disposition to mental disease. 

The temperature has been studied in chorea. In mild 
cases it is normal throughout the entire course of the 
disease. In severe cases it may be raised a degree or two, 
but any greater elevation is undoubtedly due to some other 
condition. 

Complications. — By far the most frequent complica- 
tions are rheumatism and heart disease. Rheumatism, if 
present, is discovered easily enough both by the fever and 
by the painful swellings ; but it is well to remember that the 
acute rheumatism of children is often a very much vaguer 
disorder than the acute rheumatism of the adult. If pain 
is much complained of in any case of chorea the joints 
should be examined carefully. Heart disease is the com- 
plication most to be feared. The heart should therefore 
be examined frequently and carefully. Mitral regurgita- 
tion is by far the most frequent form of cardiac disturb- 
ance. In the statistics of the British Investigation Com- 
mittee there were 116 cases of mitral disease and only 6 
of aortic disease. Gowers found but two instances of aortic 
regurgitation among 252 cases of chorea. Sinkler found 
cardiac murmur in 82 of 279 cases, but he does not decide 
how many were due to organic cardiac disease. It may 
often be difficult to determine this question, but if a patient 
whose heart was normal develops a murmur while under 
observation the probability of organic lesion is very great ; 
yet since anasmia is very frequent in cases of chorea, we 
must allow, in judging cardiac conditions, for the possibility 
of haemic murmurs and slight dilatation of the heart. 
Brown and J. K. Mitchell have described patients covered 
with subcutaneous nodules. These have a more direct re- 
lation to the rheumatic fever than to the chorea.* 

An excess of urea and of phosphates has been found in 
the urine of choreic patients. It is questionable at best 

* Osier is of the same opinion ; he considers this condition a great rarity in this 
country. 



CHOREA. II y 

whether they are not in some way the result of the inces- 
sant restless movements. Convulsive attacks are referred 
to as a complication by several authors. These are not of 
a typical epileptic character, but appear to be half-choreic 
and half-spasmodic movements. Mitchell and Burr have 
recently reported a case of this sort. The cases in which 
epilepsy is associated with chorea are more than likely cases 
of organic brain lesion, in which both the hemichorea and 
the epilepsy are symptoms of one and the same process in 
the cortex. 

Duration. — It is difficult to give any accurate informa- 
tion with regard to the duration of chorea, as the disease 
can hardly be said to be ended if upon the slightest prov- 
ocation another attack sets in. A single attack may last 
from a few weeks to many months. The average duration 
is generally considered to be about ten weeks. In my own 
cases the duration of attacks varied between four and twelve 
weeks. Two and three attacks are much more common 
than a single attack. I found among 104 cases which were 
analyzed for this purpose that 50 cases had one attack; ly 
cases had three attacks ; 26 cases had two attacks ; 7 cases 
had four attacks ; 3 cases had five attacks, and 1 case had 
eight attacks. Notwithstanding this tendency to relapses 
the disease is an eminently curable one. It is only in a few 
cases that the disease becomes chronic, as in a patient of 
Meldner, who developed chorea in early life and remained 
choreic until his death, at the age of sixty-six years. 

The interval between the relapses is also subject to great 
variation. In a few cases the relapse may set in after sev- 
eral weeks ; in others after several years ; and in the case 
of chorea of pregnancy we often find that ten years or more 
have elapsed since the preceding attack. The female sex, 
for reasons too evident to mention, is more prone to re- 
lapses than the male sex. The second and third attack is 
generally supposed to be milder than the first, but there 
are exceptions to this rule, for the very worst cases of 
chorea that I have ever seen have been in patients who 
were passing through second and third attacks. Later 
attacks, as a rule, simulate the earlier ones. If the first 
attack has been a hemichorea it is very probable that 



Il8 THE NERVOUS DISEASES OF CHILDREN. 

later attacks will be of the same character. The severity 
of development, with the exceptions just mentioned, is very 
much as in the first or in the earlier attacks. 

DIAGNOSIS. — The diagnosis of chorea rests entirely upon 
the character of the movements. These are, as a rule, un- 
mistakable, and are so typical that when they occur in con- 
nection with other diseases we speak of such movements as 
" choreic " or " choreiform." In practice the question is 
most frequently raised whether a child is suffering merely 
from general nervousness or from typical chorea. It has 
been my habit to decide this point not merely upon the 
character of the movements, though it would be safe enough 
to do this, but chiefly upon the presence of other symptoms, 
which I consider of still greater diagnostic importance. I 
refer particularly to the characteristic movements of the 
tongue, and to what I have previously alluded to as the 
" facies " of chorea. In rare instances a child may be able 
to imitate the choreic movements of others and thus simu- 
late true chorea, but if it be mere simulation the attempt 
will not be a prolonged one nor will it be successful. Hys- 
terical chorea can be distinguished very readily from the 
chorea of Sydenham by the more rhythmical character of 
the movements, by the peculiarity of the onset, by the 
longer free intervals between the attacks of twitching, by 
the longer duration of the disease, and by the presence of 
other stigmata of hysteria. 

It is not generally appreciated that the choreiform 
movements associated with infantile cerebral palsies are 
apt to be mistaken for true chorea. This post-hemiplegic 
chorea is very similar to the ordinary form, but it is more 
strictly unilateral ; it is more persistent, and it is invariably 
associated with other symptoms which prove the previous 
existence of paralysis. The difficulties of diagnosis are in- 
creased by the fact that in every case of severe chorea 
there is more or less weakness of the affected members, but 
in such cases I would advise examination for the existence 
of contractures and for increase of the reflexes, symp- 
toms which are characteristic of preceding paralysis, even 
though there be little actual weakness at the time. Rigid- 
ity and increased reflexes, moreover, are never present in 



CHOREA. II9 

cases of uncomplicated functional chorea. As this post- 
hemiplegic chorea is as much the expression of organic 
lesion of the brain as is post-hemiplegic epilepsy, it is nat- 
ural that the choreic symptoms should continue as long as 
the cerebral lesion which has given rise to them continues 
in force. In a collection of cases of infantile cerebral pal- 
sies I have found this post-hemiplegic chorea present in 
about six per cent, from which it is evident that choreic 
movements are not nearly so frequently found in conjunc- 
tion with these cerebral diseases as athetoid movements 
are. I have been consulted a number of times for persistent 
chorea, and in several such cases it has been my experience 
that the family physician has overlooked a preceding hemi- 
plegic attack which could have been determined readily 
enough if attention had been paid to the existing contract- 
ures and to the increase of the reflexes. I would urge that 
in every case of chorea a careful examination be made for 
other evidences of organic brain trouble. 

Confounding chorea and epileptiform convulsions is 
scarcely conceivable, for the convulsive movements of epi- 
lepsy come on at rarer intervals, there is generally some 
momentary loss of consciousness, and there are other symp- 
toms pointing to epilepsy. In a previous publication I re- 
ferred to -the child of a colleague who would make sudden 
and very quick twitchings of an arm and of a leg. If these 
twitchings occurred while the child was walking or running 
across the room it would stand still, evidently surprised by 
these movements. It was natural to think of petit mal, 
but the frequency of the movements, the character of the 
twitchings, and the general choreic behavior of the child 
helped me to exclude petit mal and to recognize the case 
as one of true chorea. The diagnosis was corroborated by 
the very prompt result of antichoreic treatment. 

Morbid Anatomy and Pathology. — In considering 
this part of the subject we meet with very much the same 
difficulties which we encountered with regard to epilepsy, 
and the resemblance between the two is also a close one in 
this respect, that we not only have a general functional dis- 
ease, which in the one case we call epilepsy and in the other 
chorea ; but, like epilepsy, chorea is also frequently enough 



120 THE NERVOUS DISEASES OF CHILDREN. 

the expression of actual cerebral disease. It is natural 
therefore to infer that ordinary chorea must be due to dis- 
turbances similar to those which we find in cases of organic 
lesion. Almost every conceivable change in brain structure 
has been at one time or another held responsible for the de- 
velopment of chorea. See collected 84 cases of chorea on 




Fig. 47. — Dilatation of Blood-vessels in the White Matter of the Convolutions of a very- 
Chronic and Severe Case of Chorea. (Dana.) 



which a post-mortem examination had been made. In 16 
no changes were found in the central nervous system, in 32 
there were lesions in the brain and in the nerve-centres ; in 
the remainder there was congestion of the serous mem- 
branes. Ogle, Pye-Smith, and others refer to a hypersemia 
of- the brain and cord. As long ago as 1868 Steiner report- 
ed upon a careful examination of three cases of chorea. He 
found cerebro-spinal anaemia and some connective-tissue 
proliferation in the upper part of the spinal cord ; conse- 



CHOREA. 121 

quently he considered chorea to be the result of spinal irri- 
tation. Meynert and Elischer found hyaline degeneration 
in the nerve-cells of the central ganglia. The latter author 
also found changes in the vessels of the central ganglia as 
well as extravasation of blood into the connective tissue of 
the brain, and also numerous emboli in the smallest vessels 
of the cortex. He described peculiar corpuscles — highly 
refractile bodies — but Wollenberg has found them in the 
brains of non-choreic patients. Flechsig has found hyaline 
changes in the anterior divisions of the lenticular nucleus. 
More recently Dana has observed not only a general hyper- 
asmia of the brain, but a defeneration in the walls of the 
blood-vessels, in the white substance of the brain, and con- 
siderable perivascular exudation with an accumulation of 
leucocytes. Many others, chief among them Hughlings 
Jackson, have insisted upon the embolic origin of chorea, a 
theory that would be plausible enough since Dickenson 
has found that in 17 of 22 fatal cases endocarditis was 
associated with the chorea ; and yet this theory will not 
explain that large number of cases in which there is no 
involvement of the heart. Furthermore, an examination of 
the brain in fatal cases of chorea by competent observers 
has failed to reveal the presence of emboli. This view of 
the relation between capillary embolism of the brain and 
chorea was suggested by Angel Money, who noticed after 
injections of a fluid into the carotids of animals movements 
closely resembling those of chorea, and this condition was 
found after death to be associated with capillary embol- 
ism of the brain and cord. 

Lockhart-Clarke found changes in the nerve-elements 
and connective tissue in the spinal cord ; Garrod speaks of 
an overgrowth of connective tissue in the nerve centres, 
and thus we might go on quoting as many different find- 
ings as there are authors who have written upon this sub- 
ject. One of the later contributions to this subject is by 
Anton, who found a lesion or old scar in the outermost 
division of the lenticular nucleus. As the same lesion was 
present in both halves of the brain, and the chorea was 
also a symmetrical one, the author is inclined to attribute 
the choreic disease to these lesions. By way of contrast 



122 



THE NERVOUS DISEASES OF CHILDREN. 



this same author reports the case of a man, sixty-five years 
of age, in whom spontaneous and associated movements 
were entirely wanting in the left half of the body. In the 
brain of this man the thalamus was very considerably dis- 
eased, and was supposed to be the cause of the defective 
movements. I need not discuss the author's theory at- 
tributing the excessive movements to the disease of the 
lenticular nucleus, and the defective movements to the 
thalamus. Other authors, basing their conclusions upon a 
number of autopsies, have attributed choreic movements 
to disease of the thalamus. 



In an able article on rabies Golgi refers incidentally to his studies on 
cortical changes in chorea, which were published in 1874, and have been 
quoted since by v. Ziemssen 
and others. As a matter of 
historic interest the annexed 
figure is reproduced, showing 
the changes in the nerve- 
cells of the cerebellum ; Gol- 
gi also reports that the gan- 
glion cells of the cortex, and 
the cells of Purkinje, in the 
cerebellum, were calcified. It 
is doubtful, surely, whether 
there is any causal relation 
between these changes and 
others pointing to a chronic 
interstitial encephalitis, and 
the disease proper. More- 
over, Golgi's patient died at 
the age of thirty-two years, and 
in him chorea was associated 
with chronic mental disease. 

Up to the present time the results of bacteriological research are not very 
promising. Berkeley found the staphylococcus pyogenes aureus in cultures 
from the blood of a fatal case of chorea. 

Dana has published the history and autopsy of a case of chronic chorea. 
The patient was thirty-four years of age at time of death ; he had his first 
attack of chorea at fourteen, and repeated attacks after that. The post- 
mortem findings included a chronic lepto-meningitis of the convexity of the 
brain, hyaline bodies in the brain cortex, slight meningitis of the upper part 
of spinal cord, and slight meningo-encephalitis. Diplococci were found in 
the proliferating tissue between the meninges and the brain. 

The case is of unusual interest, showing that choreic symptoms may be 




Fig. 48.— Changes in Purkinje's Cells in Chorea ; 
Varicose Swelling of the Nerve-processes. (Golgi. ) 



CHOREA. 123 

associated with a wide-spread affection ; but the true pathology of chorea can- 
not be made out in any case of fourteen years' standing, nor can such a case 
be relied upon to prove the " germ-theory of chorea."* 

The only just inference from the preceding account is 
that the accurate pathology and morbid anatomy of chorea 
are still unknown. Of the changes that have been reported 
by various writers, many, if not most of them, are secondary 
and not primary. All that we can claim at present is that 
there is considerable change in the gray matter of the 
central nervous system ; that the entire motor tract may be 
involved, but that the changes occur more frequently in the 
cortex than in other parts of the brain. These choreiform 
movements are often associated with gross lesions in the 
cortex ; they are for this reason more common in the child 
than in the adult, and a lesion anywhere in the brain so 
situated that it cuts off cortical impulses may give rise to 
chorea. The occasional development of mental symptoms, 
the association of chorea and epilepsy, the one following 
upon the cessation of the other, are the symptoms which 
not only indicate a cerebral but also a cortical origin. 
With more recent methods of examination, such as those 
described by Golgi and Cajal, we may be able to make out 
the permanent changes in the nerve-elements of the brains 
of choreic persons, but even such changes may be second- 
ary to alteration of the blood-supply ; in fact the tendency 
of the present day appears to be to regard chorea as due 
primarily to vascular changes, and such vascular changes 
may be the result of infection. 

Prognosis. — Complete recovery is the rule in the ma- 
jority of cases of chorea. The prognosis may be a little 
doubtful in regard to the recurrence of the disease and the 
duration of an attack. A child that has once had chorea 
has acquired a distinct predisposition to the disease, and 
often an occurrence which would leave no impression upon 
a healthy child's nervous system is sufficient to bring about 
a relapse of the disease. Later attacks are generally as 

* Osier refers to the bacteriological researches of Pianese (Naples, 1893). Animals 
inoculated with a culture of bacillus taken from a choreic patient died " with muscular 
twitchings and convulsions," and the same bacillus could be obtained in pure cultures 
from the central nervous system. But it is doubtful whether these animals had chorea. 



124 THE NERV&US DISEASES OF CHILDREN. 

mild as the first, and there is no special reason to fear an 
unfavorable issue in later attacks unless severe complica- 
tions set in. Under such circumstances not the chorea, but 
the complicating rheumatism, or endocarditis, is the actual 
source of danger. Death occurred in only two per cent, of 
the cases collected by the British Medical Investigation 
Committee, and Sinkler states that in Philadelphia, in 
seventy-four years, there have been but sixty-four deaths 
from chorea. This latter statement does not mean very 
much, as the majority of the cases of chorea ending fatally 
would be reported as cases of one of the complicating con- 
ditions. 

There is no way of predicting positively the duration of 
a choreic attack. The milder an attack at the beginning 
the more likely it is to run a short course, whereas the 
severer forms are apt to be much more chronic, but severe 
cases under proper treatment will yield much more quickly 
than mild cases that are handled improperly. Under com- 
petent medical care the first attack may be recovered from 
in a period varying from four to ten weeks unless serious 
complications arise. A few get well more quickly, but they 
are the exceptions rather than the rule. 

Treatment. — A severe case of chorea puts the skill of 
the attending physician to a severe test, while in a mild 
case the less medical interference the better for the child. 
Increased experience, both in private and in dispensary 
practice, prompts me to urge the simple plan of treatment 
which I outlined a few years ago. 

The most important factor in the treatment of chorea is 
rest, absolute rest, often to the exclusion of all other thera- 
peutic measures. Take a choreic child that has been ac- 
customed to roam about at will and put it to bed ; it will be 
a little restless for the first few days, but it will soon quiet 
down and show the great advantage of a thorough rest in bed. 
There is difficulty occasionally in carrying out this plan, for 
mothers and nurses are only too likely to be disheartened 
by the first show of resistance on the part of the child and 
its unwillingness to remain quiet ; but with a few exceptions 
this morbid restlessness on the part of the child disappears 
within a few days, and the little patient feels very much 



CHOREA. I25 

happier in bed than out. After a few days of enforced rest 
a decided improvement is noticeable. In the milder forms 
all movements cease, and in the severer forms the child is 
no longer troubled by the annoying jactations of the limbs. 
According to the severity of the case rest should mean en- 
tire rest in bed day and night. If the disease is taking a 
favorable course, after a week or two the patient may be 
taken out of bed for half an hour, an hour, or two hours, 
and then returned again for the remainder of the day. I 
have met only very few cases in which it seemed impossible 
to carry out this treatment ; but I am firmly convinced that 
it was never the fault of the little patient but always the 
fault of incompetent and unintelligent relatives. If the dis- 
ease has assumed a mild form we can endeavor to keep the 
child quiet without keeping it in bed the entire day ; a few 
hours' rest will be better than none. It will also be of bene- 
fit to the child to forbid its taking any violent exercise, such 
as running, riding, dancing, or bicycling. 

I am so convinced of the value of this rest treatment in 
chorea that I have made it a rule, even in dispensary prac- 
tice, to insist upon this point of rest ; we go to the extent 
of preferring the mothers to report to us about the child 
rather than to have the child taken out of bed and brought 
to us at short intervals. 

Next in importance to rest is a nutritious and easily di- 
gestible diet. Milk and rest will do more for most cases of 
chorea than any other two measures. The nutritious diet 
will have a peculiarly good influence upon the many cases 
that are associated with profound anaemia. 

The monotony of this special form of rest-cure can be 
varied with advantage by the use of lukewarm baths. Im- 
mersion into a cold bath, or the wet pack, with subsequent 
friction, cannot be recommended. It is better to place the 
child in lukewarm water, then reduce the temperature by 
adding cold water, and with this water, that is growing 
colder and colder, to sponge the spine thoroughly in order 
to get the effect of the dripping water upon the skin. After 
the bath the patient should be kept quiet and wrapped up 
warmly. In every case proper hygienic and dietary meas- 
ures are of far more importance than medicinal treatment, 



126 THE NERVOUS DISEASES OF CHILDREN. 

and yet we are bound to consider the various drugs that 
have been suggested for the cure of this disease. 

Among these arsenic holds the first place. Its praises 
have been warmly sung by some, while others have decried 
it as but little better than any other drug that might be 
substituted for it. Only a few years ago Dr. Seguin, in his 
remarkably lucid lectures on the various forms of functional 
neuroses, placed arsenic first and rest second in the treat- 
ment of chorea. This order I think should be reversed, as 
I have yet to see the first case of chorea that got well more 
quickly with arsenic than without, as long as it was getting 
the benefit of rest. Dr. Seguin insists that physicians, 
almost without exception, give nearly useless doses of ar- 
senic. He regards eighteen to twenty-seven drops of Fow- 
ler's solution after each meal as the really efficacious dose. 
In my own experience very few children will tolerate these 
large doses, which should at all times be given in some alka- 
line water within an hour after meals. I have always con- 
tented myself with smaller doses, varying from four to 
twelve drops three times a day, and if the cases resisted 
treatment I prefer abandoning arsenic rather than pushing 
it to the extreme which Seguin recommends. In cases of 
excessive restlessness I have been in the habit of prescrib- 
ing the arsenic, together with the elixir of the bromide of 
sodium, or if the sleep is disturbed I give the evening dose 
only in this way and administer the arsenic alone during 
the day ; the free use of chloral and bromides is to be con- 
demned. If one must use any drug in the cases of chorea, 
arsenic is to be preferred ; but it cannot be expected to per- 
form miracles, and we must not regard it in any sense as a 
specific therapeutic agent. 

Many other remedies have been proposed, all have been 
tried, and almost all have been abandoned. For some time 
the tincture of cimicifuga was in great favor. From fifteen to 
thirty drops, three times daily, may be administered in those 
cases in which arsenic is not well borne by the stomach. I 
have seen no good reason to resort to the use of hypodermic 
injections of arsenic, as recommended by Eulenburg and 
Hammond ; Weir Mitchell some time ago proposed the 
use of salicylates ; Simon and Legroux suggested the use 



CHOREA. 127 

of antipyrin ; and the oxides and sulphate of zinc have long 
been in popular favor in the treatment of this disease ; but 
no one, I think, would venture conscientiously to recom- 
mend any of these drugs as a specific against chorea. The 
preparations of iron and of arsenic fulfil this role better than 
any other drugs, for they at least help to tone up the gen- 
eral system. Hirt recommends the use of morphine. Even 
if this drug is as effective as it is claimed to be, its use in 
young children is not to be encouraged. In the earlier 
stages of chorea it is essential for patients to obtain sleep. 
According to the age of the child chloral in five, ten, or 
fifteen-grain doses per rectum may be given. If it is neces- 
sary to substitute another drug I would suggest the use of 
chloralamid, of trional, of veronal (three to five grains at 
bedtime), and if there is a great deal of mental excitement 
I should favor the hypodermatic use of the hydrobromate 
of hyoscine (one two-hundredths to one one-hundredth 
grain). 

In the majority of cases a heart tonic will be necessary. 
Digitalis in drop doses of the fluid extract or the tincture 
of strophanthus should be given in cases of heart weakness 
or feeble pulse. Blaud's pills, the sirup of the iodide of 
iron, various preparations of cod-liver oil, good stimulating 
wines, all these will be called for in some cases of chorea, 
but whatever else one may be induced to give the only 
matter of importance is that absolute rest shall be enforced. 

Erb has advised the use of electricity. A weak galvanic 
current may be employed safely enough and may be applied 
to the nape of the neck, and over the motor areas ; in which 
case, if given late at night, the current will help to bring on 
normal sleep. A moderate stabile current of from fifteen to 
twenty cells (about ten milliamperes) applied to the spine 
will help to allay restless movements of the body. Massage 
may be given in some cases in which the general nutrition 
is at a very low ebb and in which the circulation is poor. 

A special caution is necessary as regards the question of 
attendance at school. Every choreic child, however mild 
its attack may be, should be kept from school both for its 
own sake and for the sake of the other pupils who might 
imitate the disease. I have sometimes allowed myself to 



128 THE NERVOUS DISEASES OF CHILDREN. 

be persuaded to permit a child with a mild form of chorea 
to continue at school ; in almost ever} 7 instance I have had 
reason to regret it, for nothing is better calculated to bring 
out severe chorea than the competitive spirit that obtains 
in most schools. Periods of examination are fraught with 
greatest danger to those children who have had attacks in 
earlier life. The atmosphere of the school-room seems to 
have a depressing influence upon such children, and among 
the wealthier classes far better progress can be made in the 
ordinary studies if the child is instructed at home than if it 
is taught at school. It is necessary for the physician to 
take a firm stand on this question or else his treatment of 
the case will be thoroughly unsatisfactory. 



CHAPTER VI. 

CHOREIFORM DISEASES. 

HEREDITARY OR HUNTINGTON'S CHOREA. 

This disease is, on the whole, extremely rare. In view 
of its hereditary character we must consider it in connec- 
tion with the diseases of children, although it generally 
appears about the age of thirty or forty. The disease was 
first observed by Huntington, a Long Island physician, and 
it has since been designated by his name. It appears, how- 
ever, that another New York physician, Waters, described 
the disease in a letter to Dunglison. For a long time the 
disease was little known beyond America. Of later years 
it has been observed and discussed by a number of English, 
German, and French authors. The disease runs in families 
and is spread from one community to another in connection 
with the migration of the afflicted families, so that now, ac- 
cording to Gray, there are a number of communities in 
America in which the disease is prevalent ; but, as he further 
states, there is great secrecy maintained with regard to it, 
as the affliction is looked upon as a distinct stigma resting 
upon the family. 

The disease affects several members of the same genera- 
tion, but may skip as many as it selects. The descendants 
of healthy members of a family enjoy immunity from the 
disease as a rule. In King's case, which I quote on the au- 
thority of Gray, the great-grandfather was choreic. He had 
ten children, but only four were afflicted with this disease; 
they also had children who were choreic. One of these four 
had nine children, eight of whom were healthy, but the 
ninth was choreic. The ninth had five children, of whom 
four were choreic. Of these four, three had no children, 
but the fourth had a chorea whilst he was still young and 



130 THE NERVOUS DISEASES OF CHILDREN. 

was cured of it, when again at thirty-five he passed gradu- 
ally into the typical Huntington's chorea. 

Symptoms. — The disease begins, as a rule, between the 
ages of thirty and forty years, though at least a dozen or 
more cases are on record which have begun before the age 
of twenty. Males and females seem to be afflicted with 
the disease in equal numbers. The chief symptom is a 
motor disturbance, which for lack of a better name is termed 
choreic, yet it is very different from the twitchings of 
chorea minor. The muscular movements of Huntington's 
disease are coarse and grimacing, and may be distributed 
over a large area of the body. In Sydenham's chorea the 
affection is more strictly localized. In the former disease 
grimaces and all sorts of extravagant posturing are much 
more pronounced than those which we observe in the 
ordinary chorea of children. The difference in degree of 
muscular activity is so very great that one naturally doubts 
the connection between the two diseases. As a rule, the 
movements are slight at the start, affecting the face and 
upper extremities only. In the course of years they in- 
crease in intensity, and become widely distributed over the 
entire body, until in the end every single muscle or group 
of muscles may be involved. 

The muscular disturbance is in part subject to the will, 
and patients afflicted with this disease can, if they make a 
serious effort, inhibit the twitchings very much better, and 
for a much longer period, that those suffering from ordinary 
chorea can. The motor power is, as a rule, not diminished, 
but on account of the irregularity of muscular actions it is 
extremely uncomfortable for the patient to walk about, and 
he easily becomes an object of pity or of ridicule. 

There are no sensory disturbances, and the reflexes, 
while they may vary much, are not morbidly exaggerated 
or diminished. By far the most characteristic symptom of 
Huntington's chorea is the association with it of a progres- 
sive dementia. The first sign of a mental change may be 
a simple depression which is deepened by a knowledge 
of the hereditary and incurable character of the disease. 
Under the circumstances the occurrence of suicide is not 
unnatural. The mental condition at the start varies a little ; 






HEREDITARY OR HUNTINGTON'S CHOREA. 131 

the patient ma)* be either irritable or apathetic. In the 
course of time the deterioration of all the mental faculties 
is very marked and a typical dementia is developed. Speech 
is, as a rule, thick and indistinct, sometimes nasal and con- 
fused. Unless some intercurrent disease puts an end to 
life these patients may linger on for many years, may be- 
come absolutely bedridden, a burden to themselves and a 
torment to their families. 

The course of the disease is a very chronic one, and 
unfortunately does not tend to shorten human life. Sev- 
eral of those who have exhibited the first symptoms of dis- 
ease at the age of thirty have lived to sixty and seventy 
years. Whether cases beginning very early in life live 
as long, cannot be distinctly stated, but there is nothing 
improbable in supposing that they do. 

Etiology. — Among the causes of this disease none is 
more potent than heredity. It is distinctly a family dis- 
ease, and unlike other such diseases it does not seem to 
skip a generation. Persons with this disease can generate 
healthy children whose descendants may not be afflicted 
with this trouble. To all appearances an exciting cause is 
needed to develop the disease. In many of the cases the 
first symptoms appear upon severe emotional excitement 
or after some acute disease. In this respect the resem- 
blance between the hereditary chorea and chorea minor 
is very striking. Rheumatism does not, however, play the 
part in the hereditary form that it does in ordinary chorea. 

Differential Diagnosis. — The strong factor of hered- 
ity and the appearance at a relatively advanced age are 
sufficient to distinguish these cases from ordinary chorea. 
There is some danger, however, of confusing these cases 
with the post-hemiplegic chorea that is developed early in 
life, and this danger is all the greater if the onset of the 
paralytic symptoms is uncertain, or if the paralvsis has 
disappeared and the chorea remains. From Friedreich's 
ataxia the disease can be easily differentiated by the fact 
that in the former malady the disturbance of motion is 
truly ataxic and not choreic, and the reflexes are totally 
absent, whereas in Huntington's disease the reflexes are 
not markedlv altered. In Friedreich's disease there is also 



132 THE NERVOUS DISEASES OF CHILDREN. 

an ataxic gait, made worse by closing of the eyes, whereas 
in Huntington's disease nothing of the sort is observed. 
Some forms of hysterical chorea might be confounded with 
this disease, particularly if on inquiry the statement is elic- 
ited that a similar affliction has been observed in other 
members of the family, and it should not be forgotten that 
the hereditary trouble may be of an hysterical order. The 
examination of the patient and the general hysterical tem- 
perament, the fact that the choreic movements come on in 
the nature of attacks, and the determination of other hys- 
terical symptoms will help to distinguish one disease from 
the other. 

Pathological Anatomy. — The morbid changes in 
Huntington's chorea have not yet been definitely made 
out. Innumerable changes have been reported by various 
authors. These include pachymeningitis, hasmatoma, tu- 
mors of the dura, general atheroma, atrophy of the motor 
convolutions, increased fluid in the ventricles, foci of soft- 
ening in various parts, even in the ganglia. The most 
frequent states are pachymeningitis and changes in the 
cortical tissue. In a case of Charcot's the meninges were 
adherent in certain places, and the cortical substance was 
evidently sclerotic. But these varying conditions are 
largely secondary processes and do not in any way explain 
the true pathology of the disease. Dr. Osier summarizes 
the changes in a series of sections from the brain and cord 
which he examined as follows : " The arteries were thick- 
ened, and, in places, show hyaline degeneration, and in the 
smaller arterioles' the fatty changes were very marked in 
the fresh specimens from the cortex. The perivascular 
lymph-spaces were large, and contained leucocytes. The 
ganglion cells, in many sections, showed very slight changes, 
not more than are often seen in chronic cases associated 
with atrophy of the convolutions. There was the common 
vacuolation, and many cells seemed laden with pigment. 
The increase in the connective-tissue elements was more 
evident to the touch and upon section than microscopically. 
Sections of the pons and medulla showed no foci of disease. 
Beyond the thickening of the arteries and the shrinkage 
of the cells of the anterior cornua — probably an artificial 



HEREDITARY CHOREA WITHOUT DEMENTIA. 1 33 

change— the sections of the cord showed no important le- 
sions."* Oppenheim, Hoppe and others found dissemi- 
nated, miliary encephalitis of the cortex. Binswanger 
thinks the morbid changes not unlike those of general pare- 
sis. French authors attach great importance to the pro- 
liferation of the neuroglia of the cortex. 

Prognosis. — The prognosis of Huntington's chorea is 
grave as regards the cure of the trouble, and in view of the 
marked dementia which is associated with almost every case. 
Unfortunately, however, it is not a fatal disease, and patients 
so afflicted may live on to a very old age. 

Treatment. — As for the treatment of these cases the 
same principle should be observed as in ordinary chorea — 
rest, freedom from care, and excitement, separation from 
family, and change of climate, and possibly a course of 
arsenic treatment may be of some benefit. If movements 
are excessive and the sleep of the patient is unsatisfactory, 
the exhibition of hyoscine, in doses of one one-hundredth of 
a grain, or of trional or chloralamid, in ten- to twenty-grain 
doses, once or twice a day, is worthy of a trial. But, in the 
nature of things, every form of treatment will be simply 
palliative. 

HEREDITARY CHOREA WITHOUT DEMENTIA. 

There is another form of chorea of which a slight mention should be made 
in this connection. The disease has a distinct hereditary tendency, and is 
either transmitted direct from parent to child or from a more remote ances- 
tor. The disease appears early, beginning as a rule at about the age of 
puberty and continuing during life. It is characterized by distinct choreic 
movements of the hands and congue and the facial muscles. The move- 
ments may become so marked that they interfere with every voluntary effort, 
such as riding, sewing, buttoning of clothes, and the like. The lower ex- 
tremities do not become affected, and the general health of the patient is not 
much impaired, except that the annoyance of the disease may bring on a 
despondent feeling ; and in one case that has come under my notice distinct 
melancholy has occurred. It is distinguished from Huntington's chorea 
above all by the entire absence of symptoms pointing to dementia. The 
persons afflicted with this form remain bright throughout life and are able to 
attend to their ordinary affairs. It is furthermore to be distinguished from 
chronic chorea from the fact that there is no distinct history of an acute 
attack after which the chorea has been developed ; that the choreic move- 

* Greppin claims that the disease is due to a morbid process (not unlike an encepha- 
litis), which is developed gradually and in various parts of the brain. 



134 THE NERVOUS DISEASES OF CHILDREN. 

ments have never been as wide-spread as they are in such cases of chorea 
minor as develop into chronic chorea. They generally begin a little later in 
life, too, than the acute chorea minor does, and there is no distinct history of 
individual attacks. If the disease has once been established it continues 
without any marked remission, but also without any rapidly progressive 
changes. Some confusion might also arise with the post-paralytic chorea, 
but under such circumstances an examination into the past history of the 
patient, and for the evidences of persistent paralysis, will help to clear up the 
diagnosis. 

The prognosis is entirely favorable as regards life, but less can be said as 
regards the cure of the disease. I have placed such patients under the com- 
plete rest-cure, and have for a time obtained distinct improvement in the 
choreic movements. General tonic measures should be employed, and the 
proper feeding of the patients carefully looked after. 

HABIT CHOREA. 

It is well known that children are subject to " tricks " of movements. 
These include simple or co-ordinated movements of various muscles, gen- 
erally of the muscles of the face, of the eyes, of the shoulder, and even of the 
thighs. These " habit spasms " or " tics " may resemble chorea ; often, the 
jerkings are so violent in character as to suggest " epileptoid " rather than 
" choreic " disease. 

It is not always an easy matter to determine how the " trick " was en- 
gendered ; in some instances a true chorea has been the starting-point, the 
children keeping up some form of twitching movement after the St. Vitus 's 
dance has disappeared. Through imitation, habit chorea may be developed 
in children who have watched others with chorea minor. 

A habit chorea developed in early years is often continued throughout 
life. Among professional men (artists, literary men, and even physicians) 
such " tricks " are not uncommon, and the doubt arises whether serious ef- 
fort has been made to dispel the habit. Among the commoner forms of 
" habit chorea " are blinking, facial contortions, sniffing, shrugging of the 
shoulders, or some trick in speech or gesture. A friend of mine, now a well- 
known astronomer, has since boyhood never answered a question without 
first saying, Hm ? ah ? eh ? In former years this utterance was accompanied 
by an exaggerated raising of the eyebrows, as if to intensify the interrogation. 

Such habits cannot be condemned sufficiently in children. Parents 
should discipline children severely in order to rid them of the habit. 

If there is any peripheral condition (such as nasal obstruction or eye- 
strain) which helps to keep up the habit, the condition should be treated 
carefully. It is important also to make certain that the " habit spasm " is 
not true chorea. This can be done by examining for other symptoms of St. 
Vitus's dance. The general condition of children with some form of habit 
spasm may require treatment ; many of them are anaemic or scrofulous. 

Complex Co-ordinated Movements (Complex Tics). — In children 
as well as in adults, complex movements of a definite character are repeated 



COMPLEX CO-ORDINATED MOVEMENTS. 1 35 

at intervals, or may be continuous. They bear only a very slight resemblance 
to chorea ; but, as a matter of convenience, they may be discussed in connec- 
tion with " habit chorea." Some of them, the gyrospasms, for instance, 
occur in very young infants ; others occur in older children and are frequently 
associated with slight mental derangement. Some authors have included 
them under the heading of Imperative Movements, and have described them 
as head-shaking, head-rotating, head-banging, head-nodding, etc. These 
movements are often associated with nystagmus and with defective mental de- 
velopment, thus indicating that in some instances they may be due to organic 
changes in the brain. These peculiar disorders have been studied carefully 
by Henoch, Hadden, Gee, Peterson, and Mills, and have been referred to by 
Osier and others. 

Gyrospasms of the Head is a term applied by Peterson to peculiar ro- 
tary movements of the head in children, associated at times with strabismus 
or nystagmus. The movements of the eye are very rapid (Risien Russell) 
and may affect one eye only ; the nystagmus may be of the convergent order 
(J. Thomson). Two of Peterson's five cases were observed by him in my 
clinic. Both these cases were in young infants, eight and nine months of age 
respectively ; in one the movements came on after a fall ; in the other there 
was no history of traumatism. The chief points of Case II., as described by 
Peterson, are as follows: The child was nine months old when examined. 
Since the age of four months he had a rotary movement of the head. When 
the child was quiet, the head kept oscillating from right to left and from left 
to right at the rate of eighty to one hundred oscillations per minute. The 
movement ceased at times, particularly if the child's gaze was riveted upon 
some object. There was lateral nystagmus of the right eye, also ceasing 
for minutes at a time. 

These gyrospasms, which are evidently of a piece with head-nodding, 
head-jerking, etc., are scarcely to be confounded with epilepsia nutans. If 
developed in later years, they may constitute a habit, but if the movements 
begin in early infancy and are associated with nystagmus, strabismus, or 
idiocy, one need not hesitate to ascribe them to cerebral disease. The re- 
gion of the cranial-nerve nuclei would be the most probable seat of the 
trouble ; and the lesion must be supposed to be irritating in character. 

The chief etiological factors appear to be rickets, intestinal irritation, and 
dentition. The prognosis is good on the whole, except when these move- 
ments are associated with idiocy. A mild course of bromides has proved 
satisfactory in the majority of cases. Mills recommends, in addition, the use 
of two or three minims of tincture of belladonna, or one minim of the fluid 
extract of conium. 

Imperative movements associated with arithmomania (repeating every- 
thing a definite number of times) are clearly the result of mental derange- 
ment, and do not seem to me properly to belong to this group of cases 



Several other motor neuroses bearing a more or less 
close resemblance to chorea minor have been described in 



I36 THE NERVOUS DISEASES OF CHILDREN. 

connection with the chief disease. While the cases may 
have a superficial resemblance to St. Vitus's dance they 
are, as a rule, of a much more explosive character. They 
occur in families with a neurotic heredity, and represent, 
on the whole, a more serious disturbance of the central 
nervous system. It is not an easy task properly to classify 
all these conditions. There has been much confusion in 
the discussion of these subjects, and no two authors entirely 
agree in the designation of these disorders. As a majority 
of them come on later in life, or at least are fully developed 
in later years, we need not in this book treat them in a very 
exhaustive manner. 

CHOREA ELECTRICA. 

It is certain that two distinct forms have been described under this term. 
Henoch includes under it a form of choreic disturbance which resembles in 
part what is commonly designated as habit spasm, but some of his cases are 
more distinctly allied to myoclonia or paramyoclonia multiplex. Bergeron al- 
ludes to violent choreic movements occurring chiefly in children between the ages 
of seven and fourteen years. Bruns considers these two forms to be identical. 
Oppenheim is inclined to label Bergeron's form as an hysterical chorea. 

The term electric chorea should, possibly, be restricted entirely to a very 
rare disease that occurs chiefly in the northern part of Italy. I have had 
opportunity to see one case of this description in Italy, and one in an Ital- 
ian woman who came to my clinic. The condition was first described by 
Dubini, in 1846, and to avoid confusion it would be as well to speak of it as 
Dubini's disease. The disease is apt to occur in boys and girls as well as in 
men and women of advanced years. It has been supposed to be due to some 
infectious agent, but the rarity of the disease, even in those districts in which 
it does occur, would seem to militate against this view. 

The chief symptom of the disease is a series of violent spasmodic move- 
ments, affecting particularly the muscles of the neck and head, as well as of 
the extremities. Some of the cases begin with movements in an arm, which 
spread to the leg of the same side, and finally involve the opposite half of 
the body, and also the trunk, neck, and head. After some months the 
choreic members of the body become paralyzed ; there is wasting of the mus- 
cles and a loss of faradic irritability. Epileptiform seizures occur, and these 
may either be partial or general. In the course of a year or more the pa- 
tient may become entirely paralyzed, and, as in the case I saw, the patient 
was confined to his bed, unable to move a limb, while the severest choreic 
jactation continued in the muscles of the neck. The majority of these cases 
end fatally within a few weeks or months. During the course of the disease 
the patient suffers a great deal from pain, has slight elevation of tempera- 
ture, but, as a rule, does not have loss of consciousness. 



MA LA DIE DES TICS CONVULSIFS. 1 37 

The disease is so rare that sufficient pathological examinations have not 
been made to warrant definite statements with regard to its nature. It is 
probable that it is a form of cerebro-spinal disease, as is indicated by the con- 
vulsions on the one hand and the paralysis with atrophy on the other. 

MALADIE DES TICS CONVULSIFS. 

This disease, also known as Gilles de la Tourette's disease, has been 
studied most carefully by Gilles de la Tourette, Guinon, Brissaud, Meige, and 
Feindel (who have written a monograph on the subject), Oppenheim and 
others. The chief symptoms are convulsive twitchings of the facial muscles 
and other regular systematic movements, explosive conditions of speech 
known as echolalia and coprolalia, and lastly, imperative conceptions and 
impulses. The disease, as a rule, appears in children between the ages of 
seven and fifteen years. It is more apt to occur in those predisposed by 
inheritance to neurotic troubles than in others. In many instances there is a 
very distinct history of heredity, the same disease occurring in succeeding 
generations. (Charcot, Gintrac, etc.) 

The first symptoms that appear are sudden and explosive twitchings 
of the muscles of the face or of the eyes. The mouth may be twisted, and 
all sorts of grimaces may constitute the early symptoms. In a case which 
I have had under observation, the boy would begin by making grimaces 
and then turn about quickly as though he was snapping at some one. 
These movements were performed in regular succession. Movements of the 
sterno-cleido mastoid, and of the trapezius are often repeated so systemat- 
ically that they seem to be purposive. The child may at the same time begin 
to spit or to blubber, and the entire combination of symptoms suggest the 
possibility of simulation or intention, particularly if jumping or leaping move- 
ments are associated with the other symptoms. Whatever the symptoms 
may be that have been developed in a given case they are persisted in with 
remarkable regularity, and can be distinguished from intentional movements 
by the fact that the patient is evidently surprised by the suddenness and vio- 
lence of the movements. Smacking, hissing sounds are sometimes heard, 
but none of the symptoms is more characteristic than the habit of repeating 
words or sounds that are heard (echolalia), or the involuntary sudden explo- 
sive utterance of foul language (coprolalia). A little patient of mine uttered 
the worst curses I ever heard, which she had evidently picked up on the 
street, but would regret the utterance the very next moment after they had 
passed her lips, and would contritely declare that she was entirely irrespon- 
sible for the same. 

The symptoms frequently continue for years, and while the intelligence of 
the person so afflicted does not show any deficiency only a few are able to 
become masters over these explosive seizures. In some an attempt to con- 
quer the disease produces great restlessness and general excitement. In 
others the symptoms become manifestly worse under the effort to control 
them, and it is advisable under such conditions to divert the attention of the 
patient from his symptoms as much as possible. 

Imperative conceptions are frequently associated with the motor symp- 



I38 THE NERVOUS DISEASES OE CHILDREN. 

toms, and usually seem to represent a psychic explosion, the equivalent of the 
physical symptoms. Some of the children feel compelled to utter words in a 
definite sequence, to pronounce certain letters in a peculiar way ; rolling the 
" r " and making a hissing " s " are particularly frequent. In others impera- 
tive actions, such as occur in connection with neurasthenic disorders, are quite 
common. They are compelled to do their sums over and over again, to re- 
trace their steps, to pick up everything they see lying on the street or in the 
room, and are compelled to be in a state of continued activity in consequence 
of these imperative impulses. 

The course of the disease is extremely chronic ; by some it is considered 
to be absolutely incurable. But this is too extreme a view, since some of the 
cases get well. 

The prognosis should, however, be studied carefully in each case, as there 
is no telling in advance whether the patient will respond favorably or not to 
the treatment. The resemblance between this disease and other choreiform 
neuroses is at times so close a one that a differential diagnosis becomes ex- 
tremely difficult. It can hardly be confounded with ordinary chorea since 
the movements are much more systematic and more intermittent than they 
are in St. Vitus's dance. Moreover, echolalia and coprolalia never occur in 
ordinary chorea. The difficulties of speech in chorea are the very reverse of 
the explosive speech in this maladie des tics. Hysteria and hysterical chorea 
may bear a superficial resemblance to this disease ; but, on the one hand, the 
stigmata of hysteria are generally wanting, and in hysteria there is no such 
constant repetition of more or less convulsive movements as there is in this 
disease. There is, no doubt, a close resemblance between the maladie des 
tics and the jumpers and many other forms of disease which are differently 
designated according to the peculiar variety of muscular action. The one 
point, however, which the maladie des tics has in common with other similar 
conditions is its occurrence in neurotic families. The distinction between 
maladie des tics and para-myoclonia will be evident after a discussion of the 
latter trouble. 

Treatment. — The most important factor in the treatment of these pa- 
tients is their complete isolation from all other children and from their usual 
surroundings. They should be subjected to a moderately severe discipline by 
a competent nurse or parent, and should be taught self-control as far as that 
may be possible. The mere seclusion from other persons generally serves to 
lessen the excitement and the number of explosions. If the disease has led 
to a condition of exhaustion from loss of sleep or from inanition it is well to 
secure quiet during a considerable part of the entire day. This can be done 
best by the administration of chloral or of small doses of morphine. I can- 
not see the wisdom of using chloroform, as has been suggested by some. If 
the motor restlessness is extreme, hypodermic injections of hydrobromate of 
hyoscine, in doses of one one-hundredth grain, carefully administered, may 
prove of some benefit ; the ordinary nerve-tonics, such as arsenic and quinine, 
will do little good, and it is best not to waste much time in administering 
these drugs. I am confident that if any good is to be accomplished we must 
depend entirely upon isolation, proper feeding, and the use of tonic hydro- 



THOMSEN" S DISEASE. 1 39 

therapeutic procedures. Regular gymnastic or calisthenic exercises should 
be tried and may be of advantage. 

THOMSEN'S DISEASE (MYOTONIA CONGENITA). 

In connection with the various forms of disordered movements which 
have been considered in this chapter, we may discuss in a very brief way the 
disease known by the name of the physician who gave the first thorough 
description of it as it occurred in his own family. Those who object to 
nomenclature of this sort will prefer the term Myotonia congenita, but as 
Thomsen was also a subject of the disease an exception may well be made. 
Very few cases have been described in America, and the only one which 
the author has had an opportunity of seeing was the one presented to the 
New York Neurological Society, in 1886, by G. W. Jacoby. The best 
accounts of the disease are those furnished by Thomsen himself, a descrip- 
tion of it in Leyden's work on "Spinal Cord Diseases," and the mono- 
graph of Erb, who summarized the chief points of twenty-two cases, and 
added a full account of peculiar electrical changes in this disease, for 
which he proposed the term " Myotonic Reaction. " Dejerine and Sottas, 
Schiefferdecker and others have more recently contributed to the pathology 
of the disease. 

The chief symptoms of the disease are a rigidity of the muscles (myoto- 
nic contraction), which is developed whenever an attempt is made to use 
a muscle or muscles after a period of rest. If the first difficulty has been 
overcome, the action of the muscles may be entirely normal for some period 
of time. This muscular rigidity is most noticeable in the attempt to rise 
after a person has been quietly seated for some time ; in the attempt to use 
the hands in lifting, grasping, or writing, and at times considerable difficulty 
is experienced in the movements of the tongue and of all muscles concerned 
in articulation. The impression created by a patient grasping the physician's 
hand and then not being able to let go, or by his falling to the ground and 
remaining absolutely rigid until the spasm is relaxed, is one not easily for- 
gotten. The spasms are generally limited to a few groups of muscles, but in 
some cases the entire muscular system, with the possible exception of the 
ocular and respiratory muscles, is involved. The rigidity of the muscles is in- 
creased under the influence of emotional excitement and under the influence 
of cold and damp weather. Under alcoholic stimulants the rigidity is said to 
lessen, and prolonged periods of rest undoubtedly have a favorable influence 
upon the disease. 

The disease occurs in families, and very often affects several members of 
the same family. In Thomsen's family, five generations have been affected 
by this same condition. There seems to be no distinction as regards sex, 
and the disease may come on at any period of life. A number have been re- 
corded during the first decade of life, but the disease is apt to reach its maxi- 
mum during the period of greatest muscular development, and the symptoms 
are, therefore, pronounced between the ages of fifteen and twenty-five. In 
Jacoby 's case the patient, who was twenty-four years of age at the time of 



I40 THE NERVOUS DISEASES OF CHILDREN. 

examination, stated distinctly that he could not, as a boy, take part in the 
outdoor games of his comrades on account of the stiffness of his muscles. 
He attempted to play upon the organ, but found that his hands were clumsy. 
He was not able to whistle, and even in chewing his food the muscles be- 
came stiff and rigid, simulating a condition of trismus. In this case there 
was distinct rigidity of the muscles of the eyes. 

The entire muscular system is, as a rule, well developed ; there is often an 
increase of normal volume. According to Thomsen, the more the muscles 
are used the less the spasm becomes, and he advises a very active life as pos- 
sibly the only hope for an improvement in the disease. 

There are a few objective symptoms which render the diagnosis of the 
disease easy enough. The mechanical excitability of the muscles is mark- 
edly increased, a single tap of the hammer on a muscle produces a slow, tonic 
contraction of the fibres, but the contraction is not relaxed for some period 
of time. In contradistinction to the symptoms of tetany it may be stated 
that the nerves do not show any increased mechanical excitability. There 
is, furthermore, a very remarkable change in the behavior of the muscles un- 
der electrical stimulation. Erb has proposed to speak of this as " myotonic 
reaction." The faradic excitability of the nerves is not changed, but on the 
use of a strong current the muscles innervated by the nerve will be forcibly 
contracted and remain contracted for some period of time after the current is 
broken. The direct faradic excitability of the muscles is very much in- 
creased, mild currents being sufficient to produce contractions of long dura- 
tion. The galvanic excitability of the nerves is surely not increased — possi- 
bly diminished — but the same muscular phenomenon can be observed on 
galvanic stimulation of the nerve as in the case of faradic excitation. The 
direct galvanic excitability of the muscles is increased ; the anodal contrac- 
tion is, as a rule, greater than the kathodal contraction ; opening contrac- 
tions either with the anode or kathode cannot be readily obtained. The con- 
tractions are sluggish, and are continued for some time after the elec- 
trical stimulation ceases. But the most characteristic symptom of this 
myotonic reaction is a peculiar, rhythmical, wave-like contraction which pro- 
ceeds from the kathode toward the anode. This phenomenon can best be 
observed with the use of strong currents of at least 20 or 25 milliamperes, 
and if the negative pole is placed over the tendinous end of a muscle. If 
the kathode is placed in the palm of the hand, the anode on the shoulder, a 
wave-like contraction will appear and gradually w r ork its way from the mus- 
cles near the wrist to those of the shoulder. The response to the static cur- 
rent remains normal. 

These are the chief symptoms of the disease. A few complications have 
been observed in some of the cases, among which we may specially mention 
increased mental irritability and hypochondriasis, both of which are not un- 
natural in view of the annoyance which the disease causes the sufferer. 
Epilepsy and migraine have also been recorded in connection with Thorn- 
sen's disease, but as both these diseases are to a great degree hereditary the 
association may be simply a coincidence. 

The pathology of the disease is still obscure. Dejerine and Sottas found 



PARAMYOCLONUS MULTIPLEX. 141 

no changes in the nervous system, but endorse the findings previously made 
out in muscular tissue excised from the living body. The changes noted 
were an enormous hypertrophy of the fibres, with considerable increase of 
the nuclei in the sarcolemma, and an increase of the interstitial connective 
tissue. In one case examined by Erb, there was a striking vacuolization of 
the individual muscular fibre. These histological findings do not enable us 
as yet to explain the character of the disease. Thomsen considers defective 
cortical innervation to be the prime cause, but cerebral changes alone could 
not account for all the symptoms ; these must be ascribed to disease (pri- 
mary or secondary) of the muscles or to disease of the cord. Some have 
been inclined to attribute the disease to auto-intoxication. Proof is want- 
ing ; the findings point merely to an abnormal development of the muscular 
system. 

The disease can be mistaken only for tetany, and possibly for chronic mus- 
cular dystrophies ; but as Erb suggested, several years ago, a tap with the 
percussion-hammer and a few closure contractions with kathode and anode 
upon certain muscles will suffice for a diagnosis of Thomsen's disease. 

The prognosis is favorable as regards life, unfavorable in regard to a cure 
of the disease. Much can unquestionably be done by a continued exercise of 
the will and by regular gymnastic exercise. No further treatment of the dis- 
ease is indicated. 

CONGENITAL PARAMYOTONIA. 

Eulenburg * has described a somewhat similar affection, which he terms 
" Congenital Paramyotonia." This also is a distinctly family disease which 
may be traced through six generations, the disease becoming manifest im- 
mediately after birth. The chief feature of these cases is a tonic spasm last- 
ing from a quarter of an hour to several hours, and coming on chiefly after 
exposure to cold. There is distinct weakness associated with rigidity ; the 
facial muscles may be involved as well as the orbicularis palpebrarum and 
the orbicularis oris, making speech impossible during the continuance of the 
contraction. The disease is differentiated from Thomsen's disease by the 
absence of increased mechanical excitability and by the absence of anything 
resembling a myotonic reaction. Eulenburg states that there is a distinct 
tendency to kathodal and anodal tetanus. 



PARAMYOCLONUS MULTIPLEX (MYOCLONIA). 

A short reference is necessary in this connection to the above disease, 
which bears a resemblance to electrical chorea and to tic convulsif. By 
some it has been regarded as a form of hysteria, but this is not warranted, 
for cases have been observed in which there were none of the characteristic 
signs of hysteria. It is best to restrict the term paramyoclonus multiplex 
to the disease as it was originally described by Friedreich. The chief symp- 

* Neurologisches Centralblatt, June 15, 1886. 



142 THE NERVOUS DISEASES OF CHILDREN. 

torn of the condition is a rapidly repeated clonic spasm, occurring in attacks 
and affecting individual muscles or groups or muscles, and as a rule symme- 
trical muscles are involved. The muscles of the extremities are more fre- 
quently affected than those of the other parts ; the face muscles are gener- 
ally exempt, and in this respect the disease differs markedly from other forms 
of choreic and clonic spasm. These clonic convulsions are not severe 
enough to cause actual movements of the extremities, but in a few cases sud- 
den contractions of the diaphragm have been accompanied by hiccough and 
by peculiar respiratory sounds, and in the case described by Starr the move- 
ments were strong enough to produce jactitation of the head and of the 
trunk. In contrast to some of the other diseases described in this chapter, 
the myotatic irritability is increased only a little or not at all. Electrical ex- 
citability is not altered. 

The disease may come on at any period of life between the age of pu- 
berty and fifty years. It is rare in young children, and the short account of it 
here is simply given for the purpose of showing its close relationship to the 
various forms of choreic disorders. Heredity is not a prominent factor in 
the cases which have been described, but it has been found in several mem- 
bers of one family. Mental or physical strain and emotional excitement are 
the chief causes. Cases closely resembling, yet not identical with paramyo- 
clonus multiplex have been described by Hammond and others, under the 
title of " Convulsive Tremor." 

The prognosis is serious as regards a cure of the condition, though some 
of the cases have been reported improved and a few entirely cured. In the 
treatment of the disease nothing more can be attempted than regular gym- 
nastic exercise, and the exhibition of mild sedatives, such as chloral and the 
bromides, in small doses, in case the spasms are excessive. A mild galvanic 
current is said by some to have exercised a favorable influence over the disease. 

Dana has endeavored to establish a classification of myoclonias approved 
by Lundborg. It is as follows : i. Myoclonia of Friedreich, or peripheral type 
including myokymia ; 2. Myoclonia of the functional or hysterical type ; 
3. Myoclonia of the convulsive tic type (many associated spasms, choreic 
and tonic) ; 4. Myoclonia of the " familial " type or myoclonus epilepsy — 
closely associated with the third ; 5. Myoclonia of the type of the infectious 
and symptomatic choreas. (Clark and Prout.) 



MYOCLONUS EPILEPSY. 

Unverricht has described a form of myoclonus, occurring as a family 
disease and associated with epilepsy. In addition to the general clonic 
movements, the tongue, the muscles of deglutition, and even the dia- 
phragm may be involved. The epileptic seizures may be few and far be- 
tween in earlier years, are often nocturnal ; later on epileptic attacks occur 
more frequently. Lundborg, Clark and Prout have given especial attention 
to this association disease. (See Clark and Prout's article, American Journal 
of Insanity, No. 2. 1902.) 



CHAPTER VII. 

TETANUS. 

In view of recent bacteriological researches tetanus may 
be described as an infectious disease of the central nervous 
system, characterized by continuous tonic spasms with oc- 
casional attacks of clonic movements. The muscles first 
affected are, as a rule, those of the neck and jaw. Opistho- 
tonus and lock-jaw (trismus) are the first and most startling 
symptoms of the disease. According to its origin we dis- 
tinguish between traumatic tetanus resulting from a wound, 
and idiopathic or rheumatic tetanus if the cause is unknown, 
or if, in our ignorance, we attribute it to exposure to wet 
and cold. We must take special note of the tetanus oc- 
curring in new-born children (tetanus neonatorum), and 
may make a passing reference to puerperal tetanus. There 
is every reason to believe that the tetanus bacillus, discov- 
ered by Nicolaier and developed in pure cultures by Kit- 
asato, or its toxic product (studied by Brieger chiefly) is 
the direct cause of tetanus. 

Etiology. — Tetanus is far more frequent in males than 
in females. This is particularly true of those cases in which 
the traumatic factor is evident, and is easily explained by 
the greater liability of the male sex to traumatism. The 
very fact that of 46 so-called idiopathic cases recorded by 
Gowers only 9 were in females would lead one to doubt 
the spontaneous origin of these cases. While tetanus may 
occur at any time of life, it is most frequent between the 
ages of ten and forty years. It occurs within the first few 
days of life between the fourth and eleventh days, but be- 
tween this time and the age of five years there is, practically, 
immunity. Between five and ten years the tendency to 
the disease is on the increase, and continues to increase un- 
til after the age of forty years. These statistics strengthen 



144 THE NERVOUS DISEASES OF CHILDREN. 

the impression that traumatic lesion alone, plus infec- 
tion, is the only feasible explanation of the occurrence of 
the disease. 

Colored races, and particularly those living in tropical 
countries, are affected more frequently than white people. 
This has been amply proved in regard to the population of 
India and the West Indies. The filth and uncleanly habits, 
so common among these people, should be borne in mind. 
Puerperal tetanus, which is unquestionably infective in 
character, occurs very frequently still in these same coun- 
tries, but it is becoming less and less frequent in those 
countries in which modern surgical principles prevail in 
obstetrical practice. 

The previous health of the person seems to have little to 
do with the development of the disease. The most robust 
as well as the feeblest are equally liable to the infection. 
Irregular wounds, burns, scratches, and frost-bites are more 
often the cause of tetanus than the large aseptic incisions 
made by the surgeon's knife. Tetanus has been known to 
follow the extraction of a tooth, and, in the popular belief, 
lock-jaw is much feared after slight injuries to the toes 
in paring nails, etc. With greater cleanliness among the 
masses the disease will no doubt soon diminish. Tham- 
hayn states, from a large collection of cases, that tetanus 
is developed most frequently from wounds in the hand, leg, 
foot, head and neck, arm and trunk, in the order of fre- 
quency just mentioned. Injuries to the head have often 
been supposed to be peculiarly liable to produce tetanus ; 
but as such injuries are generally the result of a fall and 
contact with the earth, as in a fall from a horse, it will be 
seen that the danger of infection is greater than in many 
other instances. The same is true of tetanus following a 
splinter which has been run under the nail, and the old- 
fashioned method of stopping bleeding by putting cobwebs 
around the bleeding part may be very largely responsible 
for the tetanus. The micro-organisms of tetanus evidently 
do not require a large abraded surface, and it is question- 
able whether, even in those cases in which tetanus comes on 
after flogging, there was not sufficient abrasion of the epithe- 
lium to permit of the invasion of these minute organisms. 



TETANUS. I45 

As a rule, tetanus is developed within a period of five to 
fourteen days after the initial injury; but, in some cases, a 
few hours are sufficient for the development of the disease, 
and in cases in which the crural nerve has been enclosed 
by a ligature, full-fledged tetanus of the severest type was 
observed at once after the occurrence. The development 
of tetanus is not rare within three or four weeks after the 
injury, but beyond such a period the occurrence of tetanus 
could not well be explained. That infection alone will not 
explain all the circumstances in connection with the de- 
velopment of tetanus will be insisted on in the section on 
pathology. 

Symptoms. — The first symptoms of tetanus, if Ave except 
vague pains at the seat of injury, and a dull headache, is 
a gradual stiffening of the muscles of the neck and of the 
jaw until the head is markedly retracted (opisthotonus) 
and the jaws are firmly clenched (trismus). While the cer- 
vical muscles are apt to be involved at the beginning the 
dorsal and lumbar apparently escape ; but retraction of 
the head is soon followed by arching of the back. As the 
disease continues the legs become rigid, but the arms es- 
cape or are but little involved. The muscles of the face 
are often affected at an early stage of the disease. The eye- 
brows are raised, the ocular fissure becomes narrow, the 
mouth is distorted, the lips press against the teeth and the 
entire x face may assume what is known as the " risus sar- 
donicus." The rigidity of the muscles is painful, and the oc- 
casional clonic exacerbations which occur tend to make the 
condition still more distressing. The difficulties of degluti- 
tion and of respiration, from spasm of the thoracic muscles 
and of the glottis, increase the agony of the patient and are 
a continuous menace to life. Epigastric pains (pains possibly 
due to spasm of the diaphragm) are frequently complained 
of. During sleep the spasm usually ceases, but no sooner 
does the patient wake up than all the distressing symptoms 
return in full force. 

The pulse is rapid and feeble. Whether vaso-motor 
spasm is the cause of this peculiarity of the pulse has not 
been sufficiently determined. The temperature varies 
considerably ; in some instances it remains normal during 



I46 THE NERVOUS DISEASES OF CHILDREN. 

the entire course of the disease, in others there is a con- 
tinuous fever of from two to three degrees. H. C. Wood 
refers to the serrated tracings of the temperature chart, 
the rise coinciding with the paroxysm, and the fall of the 
line with the interval. Occasionally the temperature rises 
to 108 or even uo° F., and according to Wunderlich this 
high temperature continues for some hours after death, 
reaching even to 1 14 F. It was formerly supposed that the 
rise of temperature was due to excessive muscular action ; 
but cases in which muscular action is greatest are not nec- 
essarily those with highest or even high temperature. 
Gowers is inclined to consider the fever to be of nervous 
origin, which is probably correct if modified to mean that 
the toxines circulating in the system exercise a special ir- 
ritating influence upon the centres in the pons and spinal 
cord, or upon the heat areas in the cortex. Thirst is a fre- 
quent and most distressing symptom of the disease, and as it 
cannot be easily quenched, on account of the difficulty of 
deglutition, it adds much to the discomfort of the patient. 
The thirst is also increased by the very profuse perspira- 
tion which sets in and is a prominent factor in many of the 
cases. 

The urine is scanty and high-colored, of high specific 
gravity, all of which can be explained by the excessive ac- 
tion of the skin. Micturition is often irregular, possibly in 
consequence of spasm of the sphincter. The bowels are, as 
a rule, constipated. 

All the symptoms are apt to increase in intensity as the 
disease progresses. The majority of cases run to a fatal 
termination in less than a fortnight, often in four to five 
days. Death occurs, as a rule, either from failure of the 
heart or from asphyxia in consequence of spasm of the re- 
spiratory muscles. In other cases exhaustion from the con- 
tinued spasms and from the inability to take food may be 
the ultimate cause of death. Cases lasting above a fortnight 
are supposed to tend toward recovery. The spasms be- 
come slighter, the clonic spasm less frequent, and all the 
symptoms gradually recede until nothing but a slight rigid- 
ity of the muscles remains. The parts last involved are first 
released, and the spasms last longest in the muscles of the 



TETANUS. 147 

neck and jaw, the very parts which were first affected. A 
single recovery seems to provide immunity against the dis- 
ease for all time. While the evidence upon this point is 
not absolutely conclusive, the fact of immunity after the 
first attack would be in keeping with what we know about 
immunity from other infectious diseases. 

Varieties. — The majority of the cases are so much alike 
that it would be quite useless to speak of variations from 
the clinical type. An exception must be made, however, 
in favor of what Rose has termed " cephalic tetanus." This 
form develops, as a rule, after injury to the head, and partic- 
ularly to the region of the face supplied by the fifth nerve. 
The special characteristics of this form are the association 
of paralysis of the face on the same side as the injury with 
tetanic spasms of the other side. In addition to this we 
find spasms of the respiratory muscles and great difficulty 
in deglutition. The behavior of the patient is very much 
like that of one suffering from hydrophobia, hence this 
form has also been spoken of as " tetanus hydrophobicus." 
There is some doubt whether injury to the peripheral nerve 
is the cause of the facial palsy. Bernhardt and Remak 
proved conclusively that there was no reaction of degen- 
eration in the nerve or muscles, and the nerve has been 
found healthy on post-mortem examination. We must 
suppose, therefore, that the facial palsy in such cases is of 
reflex origin. Some support is given to this view by the 
fact that if recovery sets in, the tetanic spasm and the facial 
palsy disappear at about the same time. 

The only other variety worthy of special mention is the 
so-called abortive form of tetanus described by Kussmaul. 
In these cases spasm of the neck muscles and trismus are 
the only symptoms, and these gradually disappear. 

Tetanus neonatorum can hardly be considered a variety 
of tetanus, for it resembles in every way the tetanus of the 
adult, but in this book it will naturally deserve some spe- 
cial consideration. 

The tetanus of the new-born child sets in, as a rule, be- 
tween the fifth and tenth days after birth, though it may be 
delayed as long as twenty days. The first symptom noticed 
in the child is difficulty in taking the nipple or in drinking 



148 THE NERVOUS DISEASES OF CHILDREN. 

from the bottle ; with every attempt there is distinct rigid- 
ity of the masseters and of the muscles about the mouth, 
which interferes with the act of sucking and swallowing. 
Other facial muscles are apt to be in a state of contracture 
leading to a distinct deformity of the face. In some in- 
stances food can be poured into the mouth by a spoon with- 
out exciting a spasm, but in many others the mere touch of 
the spoon to the lips, or contact of the food with the lips 
and mouth, is sufficient to produce a spasm. 

In the earlier stages of the disease the child is quiet un- 
less food is given; but before long the spasms come on 
spontaneously without excitation by food. Spasm of the 
respiratory muscles soon forms a part of the clinical symp- 
toms, and periodic cyanosis is often one of the early symp- 
toms of the tetanic condition. Rigidity of the muscles of 
the neck and back, typical opisthotonus, and arched back 
soon follow, and to make the symptoms thoroughly com- 
plete the upper and lower extremities become the seat of 
muscular spasms. There may be slight remissions for a 
time, but the tetanic stage becomes more and more perma- 
nent, or, if absent when the child is quiet, will surely come 
on with the merest touch in lifting the child, in trying to 
give it an enema, in washing it, etc. The temperature, as a 
rule, remains normal ; in a few instances it reaches 102 or 
103 F. 

The disease is steadily progressive. One is often de- 
ceived by slight remissions and is flattered by the tempor- 
ary good result of sedative treatment, but is only too often 
disappointed by the reappearance of all the symptoms, with 
even greater intensity. Death may follow within a period 
varying from one and a half to eight or nine days, and re- 
sults either from mere exhaustion, due to the inability to 
take food, or from asphyxia, due to spasm of the inspiratory 
muscles. 

A few cases of tetanus neonatorum get well, but surely 
only a very few. Cases with fever seem to be invariably 
fatal. In those who get well the tetanic spasm disappears 
very slowly, and Henoch states that in some instances a 
rigidity of the muscles existed three weeks after the onset 
of the disease. 



TETANUS. 



49 






The cause of this early tetanus is in every way the 
same as that of tetanus in the adult, except that the en- 
trance of the bacilli into the body occurs by way of 
the umbilical cord, the care of which has been neglected. 
In former days, and particularly in those countries in which 
obstetrical cases were managed exclusively by midwives, 
whose ideas of cleanliness were very defective, tetanus 
neonatorum was very much more frequent than it is now. 
Ritual circumcision, if performed without due regard to 
modern surgical principles, may be considered an occasional 
cause of tetanus. Bathing a child at low temperatures is 
referred to by many of the older writers as a common 
cause of tetanus. The use of a bath thermometer is said to 
have put an end to an epidemic of tetanus occurring in the 
practice of a midwife who was not able to distinguish by 
the hand alone the proper temperature of water. In view of 
the modern bacillary theory of tetanus such an origin of tet- 
anus would be rather difficult to explain ; but we must con- 
cede the possibility of other than mere toxic agents affecting 
and irritating the peripheral nerves of a newly born child. 

Pathological Anatomy and Morbid Pathology. — 
Careful search has been made by many writers for the act- 
ual lesion of tetanus, but up to the present time with few 
positive results. It has been noted that rigor mortis sets in 
with unusual promptness, some claiming that the tetanic 
spasm passes without relaxation into the post-mortem con- 
tracture. All the organs of the body have been examined ; 
no special changes have been found in them. Some writers 
have reported oedema, hypostatic pneumonia, pleural ex- 
travasation, which can be accounted for by the inter- 
ference with the circulation and respiration. The muscles 
of the body are sometimes ruptured ; some contain small 
hemorrhages, but under the microscope the fibres present 
no anomalies, though Bowman refers to occasional granular 
degeneration. It has been natural to look for changes in 
the nerve filaments in the peripheral wound which has been 
the starting-point of the tetanus. In some instances noth- 
ing abnormal has been found ; in others there has been 
neuritis, with considerable swelling of the nerve, which ex- 
tended from the periphery to the spinal cord. 



150 



THE NERVOUS DISEASES OF CHILDREN. 



In the case of tetanus neonatorum an inflammation of the 
umbilical vessels has been traced for some distance within 
the abdomen, and the peritoneal covering of the vessels has 
also been found inflamed. 

As for the central nervous system, the condition most 
frequently discovered is one of passive distention of the 
vessels, associated with minute hemorrhages ; but both these 
conditions are surely secondary to the tetanic spasms and 
not in any sense the cause of them. In the spinal cord some 
investigators have reported granular disintegration of the 
gray substance, the formation of cavities containing gran- 
ular material, and changes 
also in the large ganglion 
cells, such as were found by 
Nerlich in the motor root 
of the fifth nerve. All of 
these changes are not suffi- 
ciently distinct to represent 
the actual pathological con- 
ditions of tetanus ; they are 
secondary effects, and it is 
probable that in tetanus, as 
in other of the diseases 
hitherto considered, definite 
though the symptoms may 
be, the changes are of a 
transitory character, every trace of which disappears after 
death. It would be more important, therefore, to refer to 
the morbid pathology. 

The pathology of tetanus can be readily understood in the 
light of modern researches if we regard the spasms of tet- 
anus as the result of increased irritability of the convul- 
sive centres in the brain. This increased irritability is di- 
rectly due to the influence of germs or germ products 
introduced into the system from some external injury. 
The tetanus bacillus (Nicolaier) is a rod-shaped microbe 
whose spores are attached to one end of the bacillus, which 
with its spores resembles in appearance an ordinary pin. 
This microbe is found in the soil as well as in the dust of 
dwellings. These bacilli are capable of resisting great heat, 




Fig. 49. — Tetanus Bacilli and Spores. 
(x about 1,000.) (Kitasato.) 



TETANUS. 151 

retaining their activity even after exposure to 175 F. for 
an hour (Kitasato, Zeitschrift fur Hyg., vol. vii.). Their 
spores are capable of resisting the same heat for six hours, 
but the spores are not formed if the temperature exceeds 
108 F. The bacteriologic phase of this subject has been 
studied most carefully in recent years by Ehrlicb, Wasser- 
man and Milchner, to whose writings the reader is re- 
ferred. 

As products of these bacilli we have several poisons 
(tetanin, tetano-toxin, and also tox-albumins, Brieger), which 
have been derived from pure cultures and which are ca- 
pable of exciting the disease. It is probable that the tetanus 
bacillus itself does not cause the disease, but that the poi- 
sonous substances formed in the blood by the presence of 
this bacillus are the direct cause of tetanus. It is an inter- 
esting fact that granulating surfaces do not offer a suitable 
soil for the production of these toxic substances. The 
presence of oxygen seems to prevent the penetration of the 
bacilli into the tissues underneath ; hence we can readily 
understand why scratches and hidden injuries should be 
far more dangerous than large abraded surfaces. 

Tetanus was supposed to result from direct irritation of 
the peripheral nerves ; if so, an ascending neuritis would 
■have to account for the development of the symptoms of 
tetanus. It is at present altogether impossible to deny that 
in some way or other the bacillus must have been intro- 
duced into the system even in these cases.* 

Lesions of the occiput were supposed to lead more fre- 
quently than other lesions to tetanus, but in this instance, 
too, it is more than probable that the infectious element 
cannot be disregarded. 

In no disease have bacteriological researches led to 
more satisfactory results in treatment. Tizzoni cultivated 
the virus, inoculations of which in increasing strength pro- 
duced tetanus in dogs. The blood-serum of such dogs 
destroyed the activity of the virus. He inferred the pres- 

* Since the above was written Goldscheider has given a different explanation based 
upon recent anatomical conceptions. The poison of tetanus acts upon the peripheral 
nerve-fibre. This fibre being the peripheral portion of the neuron, an increased ex- 
citability of the ganglion cell is produced, and this hyper-excitability brings about 
the tetanic contractions of the muscles with which it is connected. 



152 THE NERVOUS DISEASES OF CHILDREN. 

ence of an albuminoid body, which he called " anti-toxin," 
with which he succeeded in arresting the disease in rats. 

The now famous researches of Behring and Kitasato 
have revealed the fact that the blood-serum of tetanic ani- 
mals produces immunity in others, and that the serum of 
the animals thus rendered immune has still more active 
anti-toxic qualities. It was during the year 1894 that Beh- 
ring showed that the blood-serum of immunized animals not 
only produces immunity in others, but also has a distinctly 
curative effect. T&muffi {Centralblatt fur Bacteriologie, 1892) 
reported a successful use of anti-toxin injections in the case 
of a peasant, aged seventy-four, who had developed tetanus 
as the result of a lacerated wound of the little finger of the 
right hand. One hundred and fifty grammes of the anti- 
toxine was given in this case, when on the eleventh day 
after the beginning of the injections a complete recovery 
ensued. The blood-serum from this patient did not produce 
tetanus when injected into a rat. No doubt other success- 
ful cases of this kind will soon be reported, and there is 
much to hope from this serum therapy in the case of tet- 
anus. 

Differential Diagnosis. — The diagnosis of tetanus is 
easy in the majority of cases. It is possible to mistake 
tetanus for strychnia poisoning, but in the latter the symp- 
toms never begin with distinct trismus. The symptoms 
also develop with much greater rapidity, and the severe 
pain referred to the stomach, as well as the absence of ex- 
ternal injury, would lead one to suspect the effect of strych- 
nia. 

If there is great difficulty in deglutition the symptoms 
of tetanus may resemble somewhat those of hydrophobia, 
but the entire absence of trismus, and the fact that all the 
symptoms are excited only if an attempt is made to swallow, 
and the previous history of the case, will enable one to dis- 
tinguish hydrophobia from true tetanus. 

As for mistaking tetanus for tetany, such confusion is 
scarcely possible if the mode of onset from the periphery 
inward, in cases of tetany, is kept in mind, and if we also 
remember that no such interval exists in tetanus as in the 
lesser disease. The favorable course of the disease will 



TETANUS. 153 

render still further aid, after the lapse of a few days, in dif- 
ferentiating between tetany and tetanus. 

Prognosis. — The prognosis is invariably grave. A mor- 
tality of eighty-five per cent, does not overstate the facts. 
Other things being equal, the prognosis is the more grave 
the more thoroughly a lacerated wound has been infected. 
Recovery in a case of tetanus after compound fracture 
of the limbs is very rare. The longer the interval between 
the injury and the first onset of the symptoms the better 
the prognosis, and cases which begin after ten days usually 
take a more favorable course than those which come on 
very early after the injury. If the symptoms have lasted 
more than ten days there is some reason to expect a fa- 
vorable result. On the whole it is a disease very much to 
be feared, and a guarded prognosis is quite in order until 
a very decided improvement sets in ; but possibly the recent 
advances in serum therapy may in the course of time en- 
able us to be more hopeful. 

Treatment. — In the treatment of tetanus, prophylactic 
measures are by far the most important. There is no good 
excuse at the present day for the infection of any surgical 
wound, or if the wound has been received under conditions 
which make infection possible, thorough antiseptic measures 
should be at once employed, and even after the first symp- 
toms of tetanus have been observed the condition of the 
peripheral wound should be carefully examined into and 
everything should be done to prevent further contamina- 
tion and infection. 

If recent investigations are carried to a successful issue, 
the most satisfactory and the most rational treatment of 
tetanus will unquestionably be that by injections of anti- 
toxin or any of the substances which may hereafter be 
proved to possess the property of counteracting the poison 
of tetanus in the human system. Behring has perfected the 
anti-toxin treatment of tetanus, insisting, also, that the injec- 
tion be made as near as possible to the seat of infection. 

In the majority of cases, and particularly in those occur- 
ring at a distance from large medical centres, from which for 
years to come these anti-toxins will have to be obtained, the 
older methods of treatment will still have to be resorted to. 



154 THE NERVOUS DISEASES OF CHILDREN. 

The general management of the patient is the first im- 
portant consideration. Absolute quiet should be secured 
by darkening the room, keeping out all visitors and noises. 
Nutritious but liquid diet should be given, and given slowly, 
so that the difficulties in deglutition may not be increased. 
If the trismus is very marked a wedge may have to be in- 
serted between the teeth to secure enough space to intro- 
duce a tube through Avhich liquid can be taken into the 
mouth. If this cannot be done, the patient must be fed 
through the nose or else by the rectum. 

In former times it was considered wise to remove a 
peripheral scar, or even to amputate the part that included 
the seat of injury ; but according to our present conception 
the presence of tetanus bacilli in the system Avould prove 
that the disease was no longer a local one, and that excision 
of the lesion would not prove sufficient to bring about re- 
covery. 

Among the drugs employed in the treatment of this dis- 
ease chloroform by inhalation is unquestionably the most 
satisfactory. Woods, of Philadelphia, has recommended the 
use of nitrite of amyl, and Gowers reports that its use at 
Guy's Hospital has proved that the spasms became more in- 
tense at first but slighter afterward. Both chloroform and 
nitrite of amyl are palliative measures, but not curative in 
any true sense. Chloral hydrate has an advantage over 
the preceding drugs, inasmuch as it helps to produce sleep, 
and can be given continuously for a long period of time. 
To be effectual large doses of five to fifteen grains should be 
given at a time, and repeated several times during the day 
according to the needs of the case. 

There can be no reasonable objection to the use of opium 
and morphia in the form of suppositories, or by hypo- 
dermatic injection. Morphia may be exhibited in doses 
of one-twelfth to one-sixth grain, according to the age of 
the child, and can be given in connection with other nar- 
cotics. The use of conium and of gelsemium has been 
warmly recommended by various observers, but the results 
are not satisfactory enough to advise the general adoption 
of such measures. Curara in doses of one-fiftieth to one- 
half grain every hour has been urged by some ; but it is 



TETANUS. I 5 5 

surely a dangerous drug, and its application would be 
rather in the nature of an experiment than of safe thera- 
peusis. Continued warm baths, electricity (galvanism to 
the spine and the muscles), all these measures have been ad- 
vocated by some, but without much result. If the bromides, 
chloral, opium or morphia, and chloroform, judiciously ad- 
ministered, do not bring about a favorable result it is safer 
not to exhibit other drugs. 

In the treatment of tetanus neonatorum the same drugs 
may be employed as are recommended in tetanus of the 
adult, but the dosage should be modified in keeping with 
the age of the patient. 



CHAPTER VIII. 

TETANY. 

Tetany or tetanilla is a disease which occurs with far 
greater frequency in Europe than in this country. Osier 
and Weir Mitchell speak of its exceeding rarity in the United 
States. It occurs with about equal frequency in adults and 
children. As the name indicates, " the little tetanus " is 
characterized by attacks of tonic spasms of various groups 
of muscles, particularly of those of the upper extremities. 
It is only within recent years that the disease has been prop- 
erly studied, though the first account of it goes back as 
far as the year 1830, when Steinheim described this group 
of symptoms as a special form of articular rheumatism. In 
the following year Dance published an article in which he 
expressed the view that the intermittent character of the 
spasms proved the disease to be of a malarial order. The 
name of the disease we owe to Corvisart, who in 1852 re- 
viewed the entire subject. His observations were preceded, 
however, by those of Trousseau, who had observed the 
disease in nursing women, and assumed a connection be- 
tween tetany and the function of lactation. He therefore 
termed it " contracture rheumatismal des nourrices," but 
later on he observed the trouble in children and in adults 
with intestinal obstruction, and so abandoned the lactation 
theory. It was Trousseau who first discovered the very 
important fact that these attacks could be excited by com- 
pression of the arteries and nerve-trunks of the affected ex- 
tremity. 

Our knowledge of this disease has been enhanced by the 
observations of Chvostek, Koppe, Baginsky, Von Jaksch, 
Bernhardt, and Escherich. The most comprehensive articles 
on the subject have been written by Weiss, of Vienna, in 
1881, by Frankl Hochwart and by Escherich. 



TETANY. 



157 



Symptomatology. — The symptomatology of tetany in- 
cludes the symptoms to be noticed during the attack and 
those during the period of latency. The attack is preceded 




Fig. 50.— Position of Hands in the Spasm of Tetany. (After Oppenheim.) 

by vague tingling pains, by formication in the hands, fore- 
arms, and legs; a feeling of stiffness soon follows, and subse- 
quently the spasm of the muscles sets in. This tonic spasm 



158 THE NERVOUS DISEASES OF CHILDREN. 

occurs more frequently in the upper extremities, and gives 
rise to such a marked rigidity of the muscles that passive 
movements are for the time being impossible. The position 
of the hand during the spasm will necessarily vary accord- 
ing to the groups of muscles affected. It is a common 
occurrence for the hand to assume the shape of the 
accoucheur's hand. Occasionally also the thumb is very 
firmly pressed upon by the flexed fingers and the nails are 
buried in the skin of the palm. In some rare cases there is 
complete extension of all the fingers. The forearms are gen- 
erally flexed, and the upper arms closely pressed against the 
chest. If the lower extremities are involved the thighs may 
be adducted, the legs extended or flexed, while the toes are 
apt to assume the position of talipes equinus. The spasms 
may also affect the muscles of the abdomen, of the chest, 
and of the back. These tonic contractions of the abdomi- 
nal and thoracic muscles may interfere with the movements 
of the diaphragm, and with the respiration, causing dyspnoea 
and cyanosis. If the muscles of the neck be also involved, 
the return of the venous blood from the brain may be re- 
tarded, as reported by Weiss in one case, in which loss of 
consciousness was the result of such neck spasm. Opistho- 
tonus is not infrequent, but trismus is rare and never occurs 
in the beginning of an attack as in tetanus. In severe cases 
spasms of the ocular muscles have been observed, of the 
oesophagus, of the larynx, and of the muscular apparatus of 
the bladder (a desire to urinate but micturition impossible). 
During the attack the patient complains of severe pain in 
the affected muscles ; there is also a diminution of tactile 
sensibility in the extremities, the muscular sense is often 
deficient, and while standing on the floor patients have a 
feeling as though they were walking on velvet. 

Elevation of temperature as high as 104 F. has been ob- 
served ; but this is exceptional, as Weiss records an average 
of only one such case in twelve. 

Headache, vertigo, tinnitus aurium, and excessive perspi- 
ration are other symptoms which have been noted during 
an attack of tetany. The attacks may last only a few 
minutes, or may cover a period of hours, or even days. Se- 
vere attacks of tetany may bear a striking resemblance to 



TETANY. 159 

genuine tetanus; but it should be noted that there is no ini- 
tial spasm of the masseters in tetany, and that in this form 
the spasm spreads from the periphery inward, and not cen- 
trifugally, as is the case in tetanus. Moreover, the reflex 
excitability, is not nearly as great in tetany as in tetanus. 
In tetany the patients may be entirely free from attacks for 
hours, and even for days, whereas in tetanus the symptoms, 
as long as the attack lasts, are continuous. 

Etiology. — The disease occurs chiefly in very young 
persons. The cases observed by Koppe were all from one 
to two years of age. Ganghofner reported 40 cases ; of 
these 5 were between two and three years of age and the 
remaining 35 between the ages of one month and two years. 
Gowers tabulated 142 cases ; of these 64 occurred from one 
to four years, and 36 from ten to nineteen years of age. On 
going over the literature of the subject a few years ago, taking 
all cases of tetany into consideration, it was evident that 
the majority of cases of tetany were observed between the 
ages of sixteen and thirty-five years. At one time the disease 
was classed among the professional neuroses, but this fallacy 
was corrected by Kussmaul. Any exhausting disease may 
be regarded as a possible etiological factor, but exposure to 
cold and wet is referred to most frequently as a predispos- 
ing cause. Intestinal irritation is another cause, and may 
be associated with eclampsia and laryngospasmus, two con- 
ditions which we know are also frequently excited by in- 
testinal irritation. Riegel instances a case in which attacks 
of tetany were inhibited by the removal of the ova of tasnia 
medio-canellata and tricocephalus dispar. Weiss reports 
the occurrence of tetany as a complication of typhoid fever. 
It has also been observed together with small-pox, Bright's 
disease, malaria, chorea, and even after severe mental 
shock. The only just inference from all this is that 
tetany is liable to occur after any exhausting disease in 
those who are predisposed to this form of spasm. Weiss 
deserves special credit for bringing into prominence the re- 
lation between tetany and extirpation of the thyroid gland. 
The disease sometimes appears in epidemic form, if we are 
to credit the account of such occurrences in the schools and 
prisons of France. A similar epidemic occurrence is re- 



l6o THE NERVOUS DISEASES OF CHILDREN. 

ported by J. Lewis Smith, Escherich, and others. That it 
is much more frequent in some countries than in others has 
already been alluded to. 

The male sex is affected a little more frequently than the 
female ; Rilliet and Barthez recording 20 out of 28 cases in 
boys. In Gowers's statistics of 142 cases, 76 were males and 
66 females. Although the disease sometimes occurs in 
families there seems to me to be insufficient evidence of 
actual hereditary predisposition to this disease. 

Symptoms of the Latent Period. — In the interval 
between the attacks the patient may appear to be entirely 
well, but if examined carefully he will exhibit a weakness, 
with slight rigidity of the affected muscles. The calf mus- 
cles are commonly the seat of mild contractions. Chvostek 
observed slight contractions of the orbicularis palpe- 
brarum. 

The intervals between the attacks of tetany may vary 
in duration from several hours to a few days, and even a 
few months ; but, of course, we can speak of a latent inter- 
val only in case the disease can be proved still to exist. 
This can be done by eliciting Trousseau's symptoms and 
by proving an increased electrical and mechanical excita- 
bility of the parts affected. 

Trousseau's Symptom. — The famous French physician 
discovered that in persons afflicted with tetany a charac- 
teristic attack can be elicited by pressure upon the large 
nerve-trunks and the arteries of the extremities usually 
affected during an attack. The attack ceases as soon as 
pressure is removed. Kussmaul and Quincke maintain 
that in some cases pressure on arteries only is necessary, 
while in other cases the slightest pressure on a nerve-trunk 
is sufficient to produce contractions of all the muscles sup- 
plied by this nerve. Trousseau's symptom is absolutely 
pathognomonic of this disease. 

Increased electrical excitability has been found to be 
characteristic of tetany by Erb, Chvostek, Weiss, and oth- 
ers. According to these authors the faradic and galvanic 
responses of the motor nerves are enormously increased dur- 
ing the interval between the attacks of tetany. They were 
not only able to obtain kathodal closure contractions (K. 



TETANY. l6l 

C. C.) with very small currents, but were able with moder- 
ate currents to obtain a kathodal closure tetanus, and even 
an anodal opening tetanus, which has not been observed in 
any other condition. Chvostek and Weiss claim that this 
phenomenon is exhibited in the facial and in other periph- 
eral nerves. Erb found the electrical excitability greatest 
at the time when the attacks were most frequent. 

Increased mechanical excitability is a still more striking 
symptom of the condition, a simple touch with a percus- 
sion-hammer upon a nerve-trunk being sufficient to pro- 
duce contractions of the muscles supplied by the nerve. I 
remember distinctly the cases in the Vienna General Hospi- 
tal in which pressure with a lead-pencil upon the focal point 
of the pes anserinus was followed by contractions exactly 
like those which a strong faradic current applied to this 
part would have produced. This is by far the most con- 
venient test to make in cases in which the existence of tetany 
is suspected, and it is far better to endeavor to establish 
this fact of increased mechanical excitability than to excite 
an attack by pressure upon a large nerve-trunk or a large 
artery. 

Differential Diagnosis. — There can be but little 
difficulty in differentiating between tetany and other con- 
vulsive disorders. From tetanus it can be distinguished 
sufficiently by the intermittent and centripetal character 
of the attacks, by the absence of trismus at the beginning 
of the attack, and, above all, by the presence, during the in- 
terval, of Trousseau's symptom, and the increased mechan- 
ical and electrical excitability. I have known a case of 
frequently repeated epileptiform convulsions to be mis- 
taken for a case of tetany, but the loss of consciousness, the 
universal tonic and clonic movements, the turning of the 
eyes, the stupor following an attack, and the extreme short- 
ness of the attack, are the symptoms that will help to dif- 
ferentiate the epileptiform attack from one of tetany. 

Morbid Anatomy and Pathology. — Post-mortem ex- 
aminations of cases of tetany have -been made by Langhans 
and Weiss, but up to the present day there have been few 
positive pathological findings. Langhans claims to have 
found a periarteritis and periphlebitis of the white commis- 



1 62 THE NERVOUS DISEASES OF CHILDREN. 

sure and of the anterior horns of the cervical portion of the 
spinal cord. Weiss failed to find any such changes in his 
cases. He formulated an ingenious theory of the disease 
according to which the attacks of tetany are due to an irri- 
table condition of the gray matter of the medulla and spinal 
cord. This irritable condition is the result of sympathetic 
disturbances causing irregularities in the vascular innerva- 
tion of the blood-vessels of the spinal cord. Gowers is in- 
clined to look for the chief changes in the motor cells of 
the spinal cord, but all this is in the nature of speculative 
pathology. Schlesinger has very recently reviewed the 
various theories concerning the nature of tetany. He con- 
cludes that none of them is entirely satisfactory, while, ac- 
cording to his own ideas, tetany is a disease of the entire 
nervous system, some of the symptoms being due to an af- 
fection of the peripheral nerves ; but the tetanic spasms, 
as well as Trousseau's phenomena, are held to be the result 
of increased excitability of the gray matter of the central 
nervous system, chiefly of the brain, medulla oblongata, and 
spinal cord. Peripheral irritants of various kinds may give 
rise to vasomotor disturbances in the spinal cord, and these 
may be the cause of functional changes. This last theory 
is but a little more satisfactory than the preceding ones, for 
nothing is known as yet of that special peculiarity and 
special irritability of the central nervous system which 
give rise to this disease. Irritability of the nervous system 
is so common that something more is needed to explain 
these very unusual manifestations of the disease. That a 
zymotic factor enters into the etiology of tetany can 
scarcely be doubted, but the exact nature of such infection 
is a matter for future study. 

The occurrence of tetany after extirpation of the thyroid 
gland points the way to a future investigation regarding the 
origin of the disease. If it is the function of the thyroid 
gland to eliminate mucin from the body, it is natural to 
infer that the continuance of this toxic substance in the sys- 
tem is responsible for the tetanic spasm that sets in when 
the gland is removed by surgical interference. 

A special caution is in order not to confound ordinary 
carpopedal spasm, or any of the short clonic and tonic 



TETANY. 163 

spasms that so frequently occur in children, with true 
tetany.* The term should be restricted to those cases that 
present distinct attacks and a free interval, characterized 
by Trousseau's symptom and increased excitability. 

Prognosis. — The prognosis is favorable excepting in 
those few cases in which serious lung trouble may result 
from continued spasm of the respiratory muscles ; but the 
prognosis should be very guarded with respect to the dura- 
tion of the disease. It may vary from a few weeks to many 
months. 

Treatment. — The removal of every possible peripheral 
irritation is the first sine qua non of treatment. If intestinal 
irritation is suspected to be the cause, free purging of the 
bowels and removal of intestinal parasites are called for. A 
change of abode may become necessary, and absolute rest 
will have to be secured at any cost. 

To shorten the attack the physician will probably have to 
resort to the hypodermic use of morphine, and possibly to 
hyoscine. Weiss reports a single case in which the attack 
was inhibited by the application of ice to the back of the neck. 
Gowers, who seems to think the spasms of tetany very closely 
related to ordinary infantile convulsions, advises treatment 
similar to that employed in convulsions. Proceding on 
this basis, inhalations of nitrite of amyl and of chloroform 
may be tried. As soon as the attack is over it would be 
well to administer chloral hydrate in daily doses of about 
one-half drachm, or the bromides in doses of from ten to 
twenty or more grains per day, according to the age of 
the child. Small doses of sulfonal, or of trional, are worthy 
of trial. During the interval careful electrical treatment 
(stabile ascending currents through the peripheral nerve- 
trunks) as well as prolonged lukewarm baths should be 
given. There is little doubt but that the majority of cases 
will get well without any therapeutic measures. The chief 
duty that devolves upon the physician is clearly that of in- 
vigorating the central nervous system by the best known 
methods and remedies, in order to enable it to resist the 
ordinary irritants which produce the disease in those who 
are predisposed to it. If the child exhibits any symptoms 

* Some of the cases reported by Vaughan are not distinct cases of tetany. 



164 THE NERVOUS DISEASES OF CHILDREN. 

of rickets, cod-liver oil and iron will be the best remedies, 
and treatment, to be successful, will have to be directed 
entirely to the improvement of the child's defective bone 
development. The bromides may be administered if the 
condition is not intense enough to call for narcotics and 
opiates, and if, for some reason or other, these latter reme- 
dies should not be employed. Gowers states that a dose of 
digitalis, given at bedtime, has been found to be the most 
useful remedy for nocturnal tetany. The action of the digi- 
talis could be explained only on the theory that the blood- 
supply of the central nervous system is deficient, and that 
there is need of increased activity. 

TETANOID CHOREA AND OTHER TETANOID DISORDERS. 

Under this title Gowers refers to a case which has been under his care 
that exhibited symptoms intermediate between those of chorea and that of 
tetany. The disease ran a fatal course, but no demonstrable lesions were 
found after death. The patient was a boy, ten years of age, with a history 
of three other relatives having suffered from diseases resembling chorea. In 
the patient the symptoms began seven months before death. They consisted 
of tonic spasm, which was continuous, and varied by paroxysmal attacks of 
similar but more intense spasm. The face was involved on both sides so as to 
cause a constant peculiar smile. The tongue was pressed up against the pal- 
ate, impeding swallowing and preventing speech. The arms were extended, 
pronated, and rotated inward so as to bring the back of the forearms outward, 
w r hile the fingers were flexed at the joints, but at times were extended and 
moved slowly in a way characteristic of athetosis. The lower extremities 
were extended at all the joints, the feet being extended in talipes equino- 
varus, and the toes were flexed. The muscles of the trunk were also in- 
volved in the spasm. At first the left side was the more severely affected, 
but afterward the spasm became equal on the two sides. 

The electrical irritability of the muscles was normal, and there was no 
mechanical excitability of the nerves. There was considerable pyrexia during 
the more severe stage of the disease. The boy became thoroughly emaciated, 
and died from exhaustion. There is very little of true tetany, and still less 
of chorea in this case as described by Gowers. While I have seen no case 
exactly like this one of Gowers, it is not rare to find irregular spasmodic 
movements of all sorts that may remind one at times of chorea, at times of 
athetosis, and then again of a cataleptic condition during febrile disorders 
in children. 

Escherich, of Vienna, who has been the most recent and a most careful 
student of tetany, calls attention to the great frequency of tetany and of 
tetanoid disorders in young children. Of eighty cases of tetany which he 



TETANY. 165 

observed in the Children's Hospital, the majority occurred between the ages 
of three to thirty months and he is inclined to infer that the artificial nourish- 
ment given to these children is largely responsible for the development of 
the tetany. Fully one per cent, of all the children under three years of age 
which have been treated in Graz were suffering from tetany. It is safe to 
say that such frequency is peculiar to certain cities. It is surely very differ- 
ent from the experience of American pediatrists and neurologists. I wish 
specially, however, in this connection to allude to Escherich's conclusions 
that even more frequent than tetany are various tetanoid disorders in the 
earliest years of life. This author goes to the extent of putting laryngo- 
spasm, muscular spasms and eclampsia into a series characterized by those 
signs which would lead one to infer that all of them were tetanoid in charac- 
ter. The present writer has been on the look-out for this special combina- 
ation of conditions and he is confident that in America this combination is 
not a very common one. In only a single instance has he seen a child that 
presented the symptoms of laryngospasm that was in a condition of muscular 
contracture resembling that of tetany and that at times had distinct convul- 
sive seizures. In such cases the tetanoid character of the condition is proved 
by the occurrence of Trousseau's and Chvostek's symptoms either at the time 
of the spasm or during the period of latency. Escherich's observations 
should encourage us to look for the existence of a latent tetany. I am 
confident, however, that these conditions are far less common in America 
than they are in Austrian and French cities. 



CHAPTER IX. 

HEADACHES. 

It requires no little skill on the part of the physician to 
discover the true cause of headaches. They do not consti- 
tute a form of disease, but are symptomatic of many or- 
ganic and functional conditions. For this reason we must 
give them the very closest study. The diagnosis of the 
kind of headache in the adult is difficult enough, but it is 
doubly so in the case of children. As the headaches that 
come on in early life are frequently continued in later 
years and give rise to much suffering, it is particularly im- 
portant that an effort should be made to nip them in the 
bud. The most distressing form of headache and the one 
most stubborn in its resistance to treatment, migraine, will 
be considered in a separate section. The remaining forms 
may be classified as follows : 

Headaches due to : 

1. Anaemia and malnutrition. 

2. Neurasthenia. 

3. Transitory hyperemia, as it occurs in heart disease 
or at the period of menstruation. 

4. Gastric disturbances. 

5. Genital irritation. 

6. Ear disease. 

7. Organic disease of the brain or its coverings (includ- 
ing specific disease). 

8. The prodromal stage of acute infectious diseases. 

9. Malaria. 

10. Uraemic poisoning. 

11. Other toxic conditions. 

It was the fashion formerly to make a differential diag- 
nosis of the forms of headaches according either to the 



HEADACHES. 



67 






character of the headache or to the seat of the distribution of 
the headache. Thus frontal headaches were considered to 
be due to gastric disturbances. Occipital headaches were 
considered to be almost pathognomonic of uraemic poison- 
ing ; but he who has an opportunity of seeing a large number 
of cases of headaches in the adult, as well as in children, will 
soon convince himself that these signs are often misleading. 
I have seen frontal headaches with disease of the kidneys, 
and occipital headaches from gastric disturbances and in 
cases of specific disease, of tumor of the brain, etc. The diag- 
nosis of the kind of headache can, as a rule, be made after 
carefully examining into the general health of the patient, 



Constipation 
Caries of Incisor 



Enors of Refraction -. 



Gastric Dyspepsia \ .. 
Anaemia ) 




Anaemia 
Neurasthenia 



-;- Eye 

-— Decayed Teeth 



Pharyngitis 
Otitis Media 



Fig. 51. — Location of Head-pains. After Dana (slightly altered). Area I. Trigem- 
inus and Facial Nerve Strands. Area II. Upper Four Cervical Nerve Strands. 



and endeavoring to find what the fundamental disturbance 
may be. It will be better for us, therefore, to take the eti- 
ological conditions mentioned above, and to endeavor to 
connect with each condition a few signs that are generally 
associated with a particular form of headache. 

1. Headaches due to anaemia. These headaches occur 
frequently in children between the ages of eight and fifteen 
years ; they are sometimes frontal, generallv vertical, and 
are described, as a rule, as a dull, boring headache, and in the 
majority of cases are most pronounced early in the morn- 
ing. They are frequently attended by a slight vertigo and 
a feeling of faintness, but none of these symptoms is char- 
acteristic enough to make the diagnosis of anaemic head- 
ache unless the headaches due to eye-strain, to neurasthenia, 



1 68 THE NERVOUS DISEASES OF CHILDREN. 

or to uraemic conditions can be excluded. The pallor of 
the conjunctivae, which may be obscured by a catarrhal 
condition very frequent in children, and above all the pal- 
lor of the gums and of the roof of the mouth, and a defi- 
ciency of the haemoglobin are the signs which help to make 
the diagnosis ot anaemic headaches certain beyond perad- 
venture 

The result of treatment will also furnish corroborative 
evidence. The child should be placed at rest, fatigue 
should be avoided in every possible way, and its diet should 
be nutritious to the extreme. It is important not only to 
give nutritious food, but to exclude everything that is of 
no decided value to the child's condition. If we allow an 
excess of fruit or of sweetmeats the child will very natu- 
rally look for these rather than for meat, fish, eggs, oysters, 
which should constitute its main diet, with a slight admixt- 
ure of farinaceous substances. One to two quarts of milk 
per day, three or four eggs, a pound or more of meat will 
be all of far more benefit to the child than any number of 
drugs. They may help a little, but I am certain that it is 
only a very little; and if there is any question of the child's 
power of assimilating these drugs I much prefer to give 
them up rather than to diminish the amount of food that 
the child takes. In the treatment of children's diseases the 
practical necessity of prescribing something often puts 
physicians at a great disadvantage. The era of rational 
therapeutics has not yet fully dawned. 

These anaemic headaches will be relieved in some in- 
stances by the proper use of hydrotherapy. It is a good 
plan to bathe these children every morning immediately 
upon rising. Sponge the head and spine first with warm 
water and then with cold, allowing the water to drip from a 
little height, so as to get the effect of the impact of cool 
water. After this they should be briskly dried and allowed 
at once to dress. In the case of very weak children it 
would be better to have the child take a little nourishment, 
say a glass of hot milk, before putting it through this pro- 
cedure. In addition to this the child should be given 
regular calisthenic exercises, which help to improve the 
general condition and to develop the muscles, and to pre- 



HEADACHES. 1 69 

vent the accumulation of fat, which is not infrequent in 
children of anaemic disposition. 

The meals should be given at regular intervals, and in 
cases in which the appetite is poor the plan of giving small 
quantities at frequent intervals, say every two hours, is to 
be preferred to that of only three meals during the day. 

The most useful drug to be employed in the treatment of 
these headaches is caffeine in doses of one to two grains 
every hour, or at least three times a day. Black coffee will 
often answer the same purpose. From the combination of 
phenacetine and caffeine I have seen very little benefit, and 
it surely has no advantage over the administration of caf- 
feine alone, and is possessed of the disadvantage that phe- 
nacetine acts unfavorably upon the hearts of some children. 
If iron is to be given let it be administered in the most 
easily digested form, either in the form of reduced iron, or 
in one of the many preparations of the peptonates or albu- 
minates of iron that are now in the market. Arsenic can 
be given in small doses of from three to four drops of the 
Fowler's solution several times a day. In cases in which a 
general tonic effect is desired, we can give a palatable com- 
bination of iron and quinine with small doses of strychnine, 
from one-eightieth to one-sixtieth of a grain, three times a 
day. Why phosphorus should be recommended by some I 
cannot understand. I am certain that no one can conscien- 
tiously assert that he has seen any good effect from its use, 
and whether given in pill form or in the form of Thomson's 
solution, the effect in my experience upon cases in which I 
have known it to be tried has been absolutely nil. The 
fewer the drugs employed, and the more attention is paid to 
the hygiene and the diet of the child, the more quickly 
this anaemic condition will disappear. 

2. Headaches due to neurasthenia. These headaches 
occur so frequently in persons who are at the same time 
anaemic, that the treatment of one cannot be considered 
apart from the treatment of the other. In anaemic persons 
headaches very often do not occur until they have been 
subjected to some emotional excitement, or have passed 
through some fatiguing ordeal. These headaches can be 
recognized as due to some other cause than to anaemia 



I/O THE NERVOUS DISEASES OF CHILDREN. 

alone by the presence of other symptoms expressive of the 
neurasthenic state, such as slight tremor of the tongue and 
fingers and the exaggeration of the deep reflexes, partic- 
ularly the knee-jerks ; but above all by the very persistent 
location of these headaches on the crown of the head, and 
by the description of them as a pressure or a feeling of heat 
on the top of the head. This description of the headache 
is as characteristic for children as it is for older patients ; 
and in children emotional conditions, the strain of school 
work, the rivalry between classmates, is quite as apt to bring 
about a neurasthenic state and to cause neurasthenic head- 
aches as are the more serious struggles for existence in 
later life. 

The treatment of neurasthenic headaches may be con- 
ducted on exactly the same lines as was referred to for 
those due to anaemia. The hydrotherapeutic procedures 
and rest are by far the most important, and strychnine and 
quinine in small doses will be of far more benefit than if 
anaemia were the only cause ; but a cure cannot be easily ef- 
fected unless all exciting conditions are removed, and to that 
end the child should be taken from school, and in some 
instances a change of climate and of home surroundings 
may be necessary. If this is not sufficient a short rest in 
bed with forced feeding may be attempted, for the " rest- 
cure " plan is as efficient in children as it is in adults. 

3. Headaches due to transitory hyperaemia. Fluctua- 
tions in the blood-supply of the brain sometimes cause 
severe headaches in children. I have seen many young 
persons who after some unusual excitement or intense emo- 
tion develop severe headaches, associated either with deep 
flushing, or with marked pallor of the face and with cold 
extremities. In others, again, persistent headaches come on 
after an acute exhausting disease, in whom it must be sup- 
posed that if there is any transitory hyperaemic condition 
it is rapidly followed by a lack of blood in the vessels of 
the brain and its coverings. A chronic hyperaemic condi- 
tion is probable in children afflicted with heart disease, who 
are often subject to periods of painful headaches, and a 
similar explanation must be sought for in young girls just 
beginning to menstruate, who have severe headaches for 



HEADACHES. 171 

several days preceding and during each menstrual period. 
While such headaches are often strictly periodic they are 
very different from ordinary migraine, and can be distin- 
guished from the latter, too, by the late onset of the trouble 
and by their disappearance as soon as the menstrual func- 
tion has been properly established. 

4. Headaches due to gastric disturbance are a very 
common occurrence in children of all ages. They are 
generally frontal, sometimes frontal and vertical. They 
come on suddenly and persist, as a rule, until the gastric 
condition has been relieved. These headaches are easily 
recognized by the symptoms associated with them, viz., 
fetid breath, coating of the tongue, distress in the epigas- 
tric region, flatulency, and constipation or diarrhoea. The 
diagnosis is easily corroborated by the effects of treatment, 
a single dose of oil or of calomel being sufficient, as a rule, 
to dispel them until the next serious error in diet is com- 
mitted. 

5. Genital irritation is referred to by some writers as a 
cause of severe headaches. Seguin refers to a case of oc- 
cipital headache, the worst he ever saw, which was cured 
by circumcision. I have not seen such a case, but I do 
not wish to doubt their occurrence, and if the physician is 
convinced of the cause of the disturbance the remedy is 
close at hand. In older boys, and even in girls, headaches 
are not infrequently due to self-abuse. The general nervous 
condition of the patient under these circumstances calls 
for treatment even more distinctly than do the headaches 
which are associated with a general restlessness, with pains 
in the back, with irritability, and in severe cases with a 
tendency to mental apathy or even dementia. There is 
every reason to look for this etiological factor in any case 
of persistent headaches in children between the ages of six 
and fifteen years. The treatment of the headaches under 
these circumstances is not an easy matter, and practically 
they can be cured only by the closest watchfulness on the 
part of parent or nurse. 

6. The headaches associated with ear disease are char- 
acterized by intense pain, located either in the mastoid or 
in the temporal region, and are most frequently present in 



172 THE NERVOUS DISEASES OF CHILDREN. 

the earlier stages of the disease, before the formation of 
pus. One of the worst cases of this sort that I have ever 
seen was in a little girl, two years of age, who was un- 
manageable and showed by her movements that she was in 
great distress. Every touch on the head was followed by 
a shrill cry, and the region of the ear was so sensitive that 
the slightest touch with the finger seemed to cause intense 
agony. No treatment was of any avail until after a few 
days the pus was freely discharged through the outer canal, 
and from that moment every trace of headache seemed to 
have disappeared. The child's behavior is exactly like that 
of an adult with acute ear disease ; it is true, however, that 
this special cause of headache is often discovered only by 
the merest accident. The practitioner and the specialist 
in ear diseases will meet with these cases so often, particu- 
larly in connection with the acute infectious diseases, that 
they should be on their guard. The cure depends entirely 
upon prompt treatment of the ear condition. 

7. Headaches due to organic disease of the brain or its 
coverings should be suspected in every case in which the 
pain is persistent and strictly localized. The pain may 
vary a little according to the position of the head, but it is 
present whether the child be sitting up or lying down, and 
is always elicited by the gentlest percussion of the skull. 
The headaches constitute a most valuable symptom in the 
diagnosis of tumors of the brain or of the meninges, and 
in cases of meningeal inflammation from any and every cause. 
Thus after minor or greater injuries to the head the onset 
of intense pain, particularly at the seat of external injury, 
must lead one to suspect the development of a morbid con- 
dition at this point. If due to tumor, the diagnosis can be 
strengthened by the discovery of other symptoms, which 
are bound to arise sooner or later. These are vertigo, 
nausea, and disturbances of vision due to optic neu- 
ritis. If a traumatic meningitis is the cause of pain in 
the head, slight rigidity of the neck, and possibly an in- 
equality and immobility of the pupils, will help to prove 
the diagnosis. 

Among the organic headaches we might also include 
those due to specific disease ; but these headaches, if I may 



HEADACHES. 1 73 

trust my own experience, are relatively rare in cases of 
hereditary syphilis or in syphilis acquired at a very early 
date. They are surely not nearly as constant nor so im- 
portant a symptom as are the specific headaches of later 
years. The diagnosis should be made with great reserve, 
and only if other symptoms are present which point to an 
active syphilitic process. 

8. The headaches which mark the prodromal stage of 
acute infectious diseases often give rise to very serious er- 
rors in diagnosis, and are occasionally suspected to be symp- 
toms of cerebral tumor, or possibly of meningitis. In the 
prodromal stages of typhoid and scarlatina, and of diph- 
theria, these headaches are very common indeed ; but the 
possibility of such a cause for headaches should be enter- 
tained if they are associated with a general malaise and 
with slight rise in temperature. There is no need of dis- 
cussing the treatment of this special form. Unless one 
chooses to apply cold cloths, or an icebag, or possibly to 
give small doses of phenacetine, there is nothing else to do 
but to wait for further developments, and to treat the more 
serious disease of which the headaches constitute the pro- 
dromal stage. 

9. Malarial headaches are not observed nearly so often 
in this climate as in the more southern States, and in 
those countries in which severe types of malaria are prev- 
alent. Malarial headaches are almost invariably neuritic in 
character. The supraorbital and infraorbital points are 
painful, and the entire head may be sensitive to touch. 
There is a distinct periodicity in the development of these 
headaches, or if the headaches are continuous there are at 
least periodic exacerbations. 

In making a diagnosis'of malarial headaches I follow the 
practice of examining the spleen and the blood, and not 
contenting myself with the diagnosis unless I can prove 
the existence of enlarged spleen, or the presence of the Plas- 
modium in the blood. If such evidence is obtained, a few 
large doses of quinine — from five to ten grains several times 
a day, according to the age of the child — will prove the best 
cure. 

10. Urasmic headaches are not as frequent in children as 



174 THE NERVOUS DISEASES OF CHILDREN. 

in the adult. These headaches are generally occipital, and 
are associated with slight disturbances of vision, with ver- 
tigo, nausea, and sometimes with epigastric pain. The con- 
dition of the kidneys should be carefully determined, if 
severe headaches arise during or after any of the acute in- 
fectious diseases ; above all, in scarlatina and diphtheria, 
which are known to be followed by renal complications. 

ii. Other toxic headaches deserve special mention. 
They are not frequent, but do occur often enough to make 
it necessary to examine for this possible cause. Among 
toxic substances lead is most easily productive of severe 
headaches. The poison is apt to be taken into the system 
not only with milk that is kept in leaden jars, but with 
drinking-water passing through leaden pipes, and I have 
known it to result from chewing-gum, and all sort of vile 
sweetmeats that are wrapped up in attractive papers, the 
children licking these papers in order that they may get 
the full value of their purchase. Some years ago I saw at 
my clinic a young girl, of about fifteen years, suffering from 
headaches, and from a typical lead palsy, who had evidently 
taken the poison into her system in the course of her daily 
work, which consisted of gluing together the paper in 
which chewing-gum was wrapped. She was in the habit 
of putting her tongue to the glue and the paper instead of 
moistening the former with a sponge. 

I have reserved for the last, the consideration of head- 
aches due to eye-strain. Not that I think them the least 
frequent, but because in my opinion undue importance has 
been attached to them. The cases that are due to eye-strain 
are those in which the headaches come on after reading, or 
in studying. They may persist for some time after the 
effort is made, but frequently disappear after the effort is 
relaxed. Serious errors of refraction may be the cause of 
headaches, and of continuous headaches, even though no 
effort be made to use the eyes ; but I have seen headaches 
persist so frequently after the fitting of glasses by the most 
competent oculists that I am firmly convinced that eye- 
strain is the sole cause of headaches in relatively few in- 
stances. 

These headaches are located in the frontal region, be- 






MIGRAINE. 175 

tween and over the eyebrows (Fig. 51); in some instances 
with evident eye-strain the headache is referred to the occi- 
put. I am thoroughly in favor of giving every child the 
benefit of the doubt, and of making a careful examination 
into the condition of the eyes ; but I wish to protest against 
the excessive enthusiasm of the day which implies that if 
the slightest error of refraction is discovered in a child the 
error must be the cause of all ills. What is claimed for 
headaches is claimed under similar conditions for epilepsy 
and for chorea. 

The above considerations will convince the student that 
the diagnosis of headaches is no easy matter. To make an 
accurate, or even plausible, diagnosis of this condition is 
one of the most difficult tasks in neurology. It can be done 
properly only by a careful consideration of the general con- 
dition of the patient, of the symptoms associated with the 
headaches, and of the patient's health before and after 
the headache has been developed. 

MIGRAINE. 

Among the neuroses of early youth few are more 
troublesome or more interesting than migraine, or "sick 
headache." While the disease does not, as a rule, attain its 
full development until the age of puberty or later, it begins 
so often in the earlier years of life that it belongs very 
properly to the special subjects of this book. The symp- 
toms of the disease as it occurs in children are so very much 
like the adult form that in describing one we picture the 
other. 

Hemicrania (megrim), or sick headache, is characterized 
by occasional attacks of intense headaches, frequently unilat- 
eral, which are associated with a feeling of nausea, or with 
vomiting. The unilateral headache is by far the more im- 
portant symptom of the two, for in many cases the charac- 
teristic headache is present for months and years without 
nausea ever being associated with it, though I must record 
cases from my own experience in which periodic attacks of 
nausea have occurred in children without any headaches. 
Such attacks I have interpreted to be the equivalent of or- 



176 THE NERVOUS DISEASES OF CHILDREN. 

dinary migraine. The correctness of this view has been 
proved by the occasional occurrence of attacks in which 
both nausea and headaches were present, and by the fact 
that the ordinary treatment for migraine and no other 
helped to dispel these peculiar gastric attacks. Barring 
such occasional cases, it is better for us to consider the 
typical attack in which neither headache nor nausea is 
wanting. 

In this disease we find a number of very important symp- 
toms associated with headache. Peculiar visual disturbances 
constitute a prominent feature of the disease ; these have 
been variously described by many sufferers, and amount, 
as a rule, to a temporary and partial loss of sight during the 
attack ; or there may be every possible form of visual dis- 
turbance, from simple balls of fire to distinct figures, which 
appear as regularly in the attacks of migraine as similar 
phenomena do in attacks of epilepsy. In many cases a sim- 
ple dark spot is observed ; in others, flashes of lightning 
that surprise the patient are the first symptoms of a full- 
fledged attack of migraine. A young patient of mine would 
regularly see a bright zigzag line, which she compared to 
a distant staircase ; as soon as she seemed actually to ap- 
proach the stairs intense headaches would set in, and she 
would feel dizzy, but there was never loss of consciousness, 
and nothing resembling an epileptic attack. The patient 
would then pass through a typical attack of migraine, which 
would last for several hours, and during this entire time 
would exhibit very marked photophobia. In some cases 
other special senses are affected. Tinnitus is much more 
frequent, on the whole, than any other form of sensory 
disturbance excepting those of vision noted above. 

Disturbances of sensation in the limbs are a frequent ac- 
companiment of migraine. These sensations generally take 
the form of tingling, of pins and needles, or of burning sen- 
sations. In some instances there is a general numbness, 
very much like the numbness that precedes an attack of 
epilepsy ; the sensations, however, last very much longer 
than the sensory aura would, and can, of course, be distin- 
guished from the latter by the entire absence of any typical 
clonic movements. Motor symptoms are not present as a 






MIGRAINE. 177 

rule. As there is a close association, at times, between mi- 
graine and epilepsy it is possible that we may see cases 
every now and then in which the auras resemble very much 
those that precede an attack of migraine, but the clonic 
movements are characteristic of epilepsy. 

Temporary aphasia I have met with as an accompani- 
ment of an attack of migraine. I recall the case of a young 
girl, ten years old, who had inherited migraine from her 
mother, and who greatly alarmed her parents by the sudden 
development of aphasia in connection with an attack of mi- 
graine. The girl when spoken to was able to mumble a few 
words indistinctly, but could not find the word she wished 
to say. She was in intense pain and extremely irritable, 
but, after a good night's rest, the headache had disappeared 
and with it the aphasia. This aphasia is associated with 
right-sided hemiplegia in right-handed persons. The occur- 
rences during these attacks of migraine adhere closely to 
the physiological laws of the cortex, and we may anticipate 
what we have to say upon the pathology of the subject to 
the extent of implying that the sequence of symptoms evi- 
dently proves that the entire motor district of the brain 
must be affected by the temporary defect in its blood- 
supply. 

Vertigo is another symptom that is associated with the 
headaches, at times preceding it, at other times following 
it. The vertigo is not, as a rule, as marked as it is in Me- 
niere's disease or in some organic diseases of the brain, but 
it is quite sufficient to make the patient unsteady on his 
feet, and to give rise to a great deal of discomfort. 

The aspect of the patient varies considerably during the 
attack. In some there is distinct pallor of the face and a 
feeling of coldness in the extremities. The eyeball may 
seem a trifle retracted, the vessels of the conjunctiva may 
be engorged, and the pupils may be contracted. In other 
cases the face may be extremely flushed, the pupils dilated 
rather than contracted, and the ear distinctly reddened. 
These two distinct conditions are often present in one and 
the same attack, and, in rare instances, the one-half of the 
face may present pallor with its associated symptoms, 
whereas the other half presents a flushed condition with 



1/8 THE NERVOUS DISEASES OF CHILDREN. 

the symptoms that go with it. The latter symptoms are 
evidently of the paretic order and the former of the spas- 
tic, and both are evidences of a change in the sympathetic 
nerves. Sweating of one side of the face has also been ob- 
served, as well as retardation of the pulse during the parox- 
ysms. On examination during an attack, I have found the 
various points of the trigeminal nerve quite as sensitive as 
in the milder cases of trigeminal neuralgia. In some pa- 
tients — and this is particularly true of children— the entire 
face and head is so sensitive that the patient can scarcely 
bear to rest the head on a pillow, and I have known one 
patient who insisted on " walking off " the headache rather 
than to rest the head against anything during the parox- 
ysm. 

Gowers refers to a transitory pyrexia during an attack 
of migraine in children. I have often taken the tempera- 
ture during attacks, impelled to do so by a flushed condition 
of the face, but have never found any elevation above ioo° 
F. If a higher temperature is present I should be disposed 
to look for some other condition, say some gastric disturb- 
ance, as a possible cause both of the migraine and of the 
fever. 

Etiology. — The hereditary predisposition to migraine 
is too manifest to be denied for a single moment. Not only 
do children of mothers who are thus affected inherit the 
disease, but it also occurs in the progeny of persons afflicted 
with other grave forms of functional nervous disturbance. 
Among these epilepsy, hysteria, and hystero-epilepsy are 
the most potent sources. The disease usually sets in in the 
earlier years of life. Some begin between the fifth and tenth 
years, a fair number of the cases between ten and twenty 
years, but the majority set in between twenty and thirty 
years. 

The female sex is evidently more disposed to the disease 
than males. 

The first manifestations of migraine are generally excited 
by emotional disturbances, by overwork, or worriment. In 
not a few instances I have known the rivalry among class- 
mates to have been the final cause of the development of 
migraine ; in others, some severe gastric disturbance has 



MIGRAINE. 179 

been the starting-point of the entire trouble, but it should 
be remembered that, if a predisposition did not exist, the 
derangement of the stomach might have produced a tem- 
porary headache, but would not have started a series of 
attacks of migraine. After the first attack an interval of 
some weeks, or months, may intervene before a second 
seizure takes place ; but some form of periodicity is soon 
established in the majority of the cases, and in many, even 
in young children, the attacks are apt to return at stated 
intervals — every two, every four weeks, or every second or 
third month. It is with migraine very much as it is with 
epilepsy, that the slightest disturbance of the physical or- 
ganism is sufficient to develop an attack. It is, therefore, of 
the greatest importance in migraine, as in epilepsy, that the 
most careful attention should be paid to the general hygi- 
enic and dietetic management of the child. 

Pathology. — We cannot expect to demonstrate actual 
changes in the brain, or in any other part of the central ner- 
vous system in a person afflicted with migraine, for persons 
so afflicted rarely die after a paroxysm, and the attack of mi- 
graine evidently represents a transitory change ; but since 
the anatomical basis is wanting, the opportunity for theo- 
retical speculations regarding the pathology of the disease 
is all the greater, and has been improved by numerous 
writers. There can hardly be a doubt that changes in the 
blood-supply of the brain or its coverings are primarily 
responsible for the symptoms of migraine. There is also 
reason to believe that the sympathetic nervous system is 
largely involved in this disease. According to the varying 
conditions present many neurologists are inclined to sup- 
pose that in some instances we have an angio-spastic condi- 
tion, in others an angio-paralytic state, and if we wish to 
imply our belief in the role played by the sympathetic 
nerves we can speak of " sympathetico-tonic " or " sympa- 
thetico-paralytic " forms. The behavior of the superficial 
blood-vessels in the two forms of migraine lends reasonable 
coloring to this view of the vasomotor origin of migraine. 

Some, not satisfied with this vascular theory, argue that 
there must be some inherent alteration in the nerve-cells of 
the brain, but no sufficient reason is given why, if such de- 



l80 THE NERVOUS DISEASES OF CHILDREN. 

rangement exists, there should be such violent periodic ex- 
acerbations of all the symptoms. To say that the symp- 
toms are due to a " nerve storm " is merely substituting a 
vague term for a vague conception ; yet we must acknowl- 
edge that a peculiarity in the structure or in the function 
of the cortical cells may be present in these cases, and that 
changes in the vasomotor apparatus are sufficient to pro- 
duce the phenomena of migraine in a person whose nervous 
system is thus altered, whereas the same vasomotor changes 
would be entirely insufficient to produce any such symptoms 
in persons whose brain -cells are altogether normal. It 
seems to me, therefore, that we must rely upon these two 
causes for an explanation of the disease ; surely one cause 
alone would not be sufficient to explain all the phenomena. 
We are constantly losing sight of the co-operation of forces 
and of causes that produce disease, and are hampered so 
frequently by the supposition that we must make out a 
single cause or none. 

The disturbances of vision, as well as the temporary 
aphasia occurring in some cases, prove conclusively that 
the cortical centres are involved in the disease. The visions 
of migraine can be regarded as a symptom of irritation, 
the hemianopsia as a symptom pointing to temporary paral- 
ysis of the functions of the visual centres. Whether a de- 
rangement of the cells or some other change in the consti- 
tuent parts in these centres is responsible for the loss of 
function cannot yet be proved or denied. 

The relation of migraine to epilepsy makes it also more 
probable that some primary peculiarity of the cortical cells 
is responsible for this painful affection. The resemblance 
is so close, and the sequence of the two diseases so strik- 
ing, that the two forms of disease may possibly represent 
a different degree of affection of the cortical structure. 

As for the headaches of migraine, they can be best ex- 
plained, it seems to me, on the supposition of a marked 
distention of the blood-vessels of the coverings of the brain ; 
and the fact that various points in the face are as painful as 
they are in typical trigeminal neuralgia, would lead to the 
inference that the trigeminal nerve, as it passes from the 
brain outward, is affected by this general hyperaemic con- 



MIGRAINE. l8l 

dition. The nausea and vomiting are an expression of gen- 
eral cerebral disturbances, such as is seen in cases of gross 
disease anywhere in the brain, particularly in the lower 
centres in the pons and medulla, and such as we sometimes 
find in cases of simple cerebral shock. I cannot see suffi- 
cient reason to connect these symptoms directly with an 
affection of the sympathetic nerve. 

Within the last few years much has been made of ocular 
insufficiencies as a possible cause of migraine. This matter 
has been much overdone, thanks to the labors of Dr. Stevens 
and others. As a neurologist I could pass over the rather 
elaborate discussion that has been held on this subject, 
were it not for the fact that so high an authority as Dr. 
Seguin has given his approval to this special doctrine in 
his lectures on the treatment of neuroses {New York Med- 
ical Journal, 1890). Dr. Seguin states that he has not met 
with a case of migraine in a person with normal eyes, al- 
though he has been told of two or three by oculists of good 
repute. He implies, furthermore, that if the matter has 
been overlooked in the majority of cases of migraine, it is 
because the ocular examination has not been a thorough 
one. He argues, furthermore, that the ocular origin of 
migraine is made probable by the remarkable fact that in 
many persons of both sexes the attacks diminish and then 
cease between the ages of forty and fifty years, at the 
time the power of accommodation becomes exhausted, and 
a large part of the unconscious strain which has been going 
on from early youth is removed. Seguin implies still further 
that migraine is hereditary, chiefly because ocular defects 
are hereditary. The fallacy of these arguments seems to 
me to be evident enough from the experience many of us 
have had, that the eyes are normal according to the exami- 
nation of competent oculists in many children who have 
migraine, and that the attacks continue long after the ocu- 
lar difficulty has been corrected by glasses. We are willing 
to concede that these ocular difficulties have an important 
bearing upon headaches in general, and may influence the 
occurrence of attacks in those who have inherited migraine; 
but far stronger evidence will have to be advanced before 
it will be safe to adopt Seguin's conclusions, and some other 



1 82 THE NERVOUS DISEASES OF CHILDREN. 

sufficient reason will have to be given for the action of 
mydriatics, such as belladonna, atropine, hyoscyamia, and 
cannabis indica, than the effect these have upon the accom- 
modative effort. In my own experience with the drugs 
mentioned, cannabis indica is the only one which has any 
decided effect upon the course of migraine or upon single 
attacks. 

Diagnosis. — The diagnosis of migraine is easily made. 
The early onset of the disease, the periodic attacks, and the 
character of the headaches will, as a rule, leave little room 
for doubt. It is only in those cases in which a distinct sen- 
sory aura exists that a confusion with epilepsy might arise, 
but the preservation of consciousness and the entire absence 
of clonic movements, and the duration of the single attack, 
will help to distinguish migraine from epilepsy. It may 
be a little more difficult at times to distinguish between 
migraine and headaches due to some other cause, but this 
difficulty will cease as soon as the periodicity in the occur- 
rence of the headaches has been noted, and every other 
form of headache has been ruled out by the method of ex- 
clusion. I have found some little difficulty also, particu- 
larly in young children, to distinguish between migraine 
and ordinary trigeminal neuralgia, for owing to the sensi- 
tiveness of younger patients it is not so easy to determine 
whether there are distinct painful points, or whether the 
whole face is painful, merely as a result of the fear of ex- 
amination ; but the long and free interval between attacks, 
together with the periodic occurrence of the headaches in 
migraine, will serve to distinguish this form from typical 
trigeminal neuralgia.* 

Prognosis. — The prognosis of migraine is entirely fa- 
vorable as regards life, but not so promising with reference 
to the cure of the disease. It is a matter of common ex- 
perience that a person who has inherited migraine will be 
subject to the disease for a long number of years, until it 
begins to disappear with age. It is a satisfaction, however, 
to be able to assure the patient that under proper treatment 
the attacks may be either inhibited or their severity may be 
diminished. The only grave feature about the disease is 

* Trigeminal neuralgia is a rare affection in children. 






MIGRAINE. I83 

the possible development of migraine into epilepsy, but 
even this is an infrequent occurrence. 

Treatment. — In the treatment of migraine two distinct 
objects must be kept in view. First — the cure, if possible, 
of the disease ; secondly — the amelioration of the attacks. 

The disease often defies the skill of the most experienced 
practitioner. As in epilepsy, no effort should be spared 
to accustom the patient to regular hygienic and dietetic 
habits. It is important that the child thus afflicted should 
sleep in a well-ventilated room ; that it should have regular 
and moderate physical exercise, a point of the greatest im- 
portance in those who have inherited gouty or lithasmic 
tendencies. The child should be relieved also, as far as 
possible, of all mental and physical strain. While the dis- 
ease is at its worst the patient should be kept from school, 
and all close application to study should be avoided. If it 
can be shown that the effort of reading or of studying helps 
to bring on an attack, even study at home and reading may 
have to be prohibited. As a matter of fact, however, a 
little mental occupation is often to be preferred to idleness, 
which permits the child to wait for the onset of an attack 
and often induces distinct hypochondriacal tendencies. My 
own experience has been very largely in favor of mod- 
erate employment of mind, which helps to prove to the 
child that its lot is not a worse one than that of other chil- 
dren, and helps also to avoid the idea of invalidism which is 
so frequent in children, even in those who do not for one 
reason or another care to simulate disease. 

If the child is anaemic, or if it exhibits scrofulous ten- 
dencies, iron in any of its various forms, arsenic and quinine 
in tonic doses, as well as cod-liver oil, are indicated. Phos- 
phorus is of so little use that we can disregard it alto- 
gether. 

A single attack can be relieved best by putting the 
child in bed during the period of the attack, and in a dark- 
ened room. On the whole mere rest in bed is as important 
a factor as any in the treatment of the attack. During this 
time the child should be placed on a mild diet ; an excess of 
liquids as well as of nitrogeneous food should be avoided. 
Small amounts of meat, fish, and eggs, and a moderate 



1 84 THE NERVOUS DISEASES OF CHILDREN. 

amount of milk, will answer the purpose better than if the 
patient is placed altogether upon nitrogeneous or upon an 
excess of farinaceous food. 

It is important to regulate the bowels thoroughly, and 
if the kidneys are- not very active to increase the quantity 
of urine passed. To this end some mild alkaline water, 
such as Vichy or Seltzer, can be safely recommended, 
either alone or in conjunction with milk. 

Innumerable drugs have from time to time been warmly 
recommended. Some years ago antipyrine in doses of five 
to ten grains, according to the age of the patient, was quite 
in vogue ; but I do not consider it promising enough to 
urge its use in the face of the well-known risks attending 
its exhibition. I am certain, too, that phenacetine in five to 
ten-grain doses has little or no effect. I have seen some good 
results from the combined action of the phenacetine and 
caffeine in one- to three-grain doses, but I am certain that 
the combination is not in any way superior to the use of 
caffeine alone. Caffeine is beyond a doubt one of the most 
serviceable drugs to be employed. I am in the habit of 
giving it to children under fifteen years of age in one-grain 
pills or powders, which are to be repeated every twenty min- 
utes until distinct relief is felt ; and after the first relief has 
been procured the drug should be continued in the same 
dose at longer intervals, first every two, then every three 
hours, and later on, for a period of about a week after the 
onset of the attack, it can be given safely in small doses 
several times a day, according to the condition of the 
child. Caffeine can be combined with iron, with quinine, or 
with arsenious acid. I have also had excellent results from 
a combination of caffeine and cannabis indica. The various 
preparations of the latter are so unreliable that it is as well 
to use Herring's extract (gr. -^ - ■{-). A caution is neces- 
sary, however, as regards the use of cannabis indica, for 
some patients are peculiarly intolerant toward this drug, 
and I have seen distinct symptoms of poisoning from small 
doses repeatedly employed. While the drug is not in 
reality a dangerous one, the symptoms due to the admin- 
istration of large doses are so disagreeable and are so apt 
to alarm the patient and the family that great care should 



MIGRAINE. 185 

be exercised in the use of the drug ; but if the fact of toler- 
ance on the part of the patient has once been established 
no trouble is apt to ensue during subsequent trials. Seguin 
thinks that the drug exerts a sedative, even paralyzing, 
influence upon the third cranial nerve and its attached 
muscles, including the ciliary, but the drug has as good an 
effect in cases in which the ocular apparatus has been de- 
termined to be entirely normal. 

The nitrite of amyl has been suggested on physiological 
grounds as a proper remedy, particularly in those cases in 
which there is marked pallor of the face. We might also 
expect it to act favorably, as it does in epilepsy, in those 
cases of migraine in which there is a distint sensory aura. 
The bromides and chloral help to allay the nervous excite- 
ment and to induce sleep, as do also sulfonal, trional, vero- 
nal, and chloralamid in four to fifteen-grain doses ; but I 
cannot regard them as in any sense specific remedies in 
migraine. Nitro-glycerine (one drop of the one per cent, 
alcoholic solution) has occasionally helped to diminish the 
severity of an attack. In this form it is not a dangerous 
drug, and can be safely administered under the supervision 
of a physician, of a nurse, or of an intelligent mother. The 
use of tea ana coffee in moderate quantities is strongly to 
be recommended; I have known children suffering from 
migraine to do well on black coffee who would vomit every- 
thing else that was given. I feel that I owe much to Dr. 
Seguin's suggestion that black coffee, without sugar and 
without milk, is easily digested, and that the general preju- 
dice regarding this beverage is due to the milk and sugar 
that have been invariably added, and that have given rise 
to fermentative processes in the gastro-intestinal tract. 

As for the correction of the ocular defects, I do not wish 
to oppose sensible treatment by competent oculists, but I 
do oppose treatment based upon extreme theories. I would 
have the eye condition corrected on the principle that in 
migraine, as in epilepsy, every physical defect should be 
remedied; but I must insist that such slight errors in re- 
fraction and in accommodation are not the cause of this 
neurosis. 



CHAPTER X. 

THE DISORDERS OF SLEEP. 

The healthy new-born child sleeps during the entire day 
and night, except when it is being nursed or dressed. Dur- 
ing the first month the infant is awake only three or four 
hours out of the twenty-four, falling soundly asleep imme- 
diately after nursing. By the end of the sixth month fifteen 
hours' sleep per day is a fair average, which is gradually de- 
creased until at the age of one year the healthy child sleeps 
about twelve out of the twenty-four hours. From one year 
to four years an average of ten hours' sleep should be main- 
tained, and up to the age of ten or twelve years the health of 
the child demands that it shall sleep quietly for at least 
nine hours in the day. If there is a marked departure 
from these averages, the child is either in pain or some of 
its functions are not entirely normal. In the acute fevers 
or in any febrile condition children are alternately drowsy 
and wakeful. Disturbances of digestion are also a frequent 
cause of restless sleep or of prolonged wakefulness. 

In addition to these special conditions, others cause in- 
somnia in children as in adults. Poorly ventilated and over- 
heated rooms often cause restlessness, and for this reason it 
is important that a window should be kept open in every 
bedroom during the entire night, and that the child should 
be covered as lightly as possible, according to varying at- 
mospheric conditions. If a child is sleepless in the absence 
of unfavorable conditions, we must seek some other cause 
for the insomnia. In many cases the mother or nurse has 
not been careful to engender the proper habits of sleep. 
Infants that are much pampered, that are taken up as soon 
as they utter the first cry, or that are wilfully disturbed by 
parents or nurse, soon become poor sleepers. From the 
first week of life the child should be laid down quietly and 



THE DISORDERS OF SLEEP. 1 87 

not rocked to sleep, and should not be disturbed except for 
good reasons. If the room is darkened a quiet sleep will 
come on very naturally. As the child grows older it is 
well not to excite it just before the hour at which it is ex- 
pected to fall asleep. With children up to the age of eight 
years the evening meal should be a light one, and their 
brains should not be disturbed by exciting stories. This 
should be observed more particularly in the case of chil- 
dren who exhibit nervous tendencies, or of those who have 
passed through exhausting diseases. 

The normal child does not fall asleep unless its hunger 
has been appeased. Insufficient nourishment is, therefore, 
to be suspected in cases of persistent wakefulness in young 
infants. With the change of nurse, or after weaning, a 
restless sleeper will often develop normal habits of sleep. 
If insomnia continues without apparent cause, we must sus- 
pect some graver trouble. In the earlier stages of tubercu- 
lar meningitis, in tumor, and in other painful diseases of the 
brain, sleeplessness is often one of the earliest symptoms. 

If the cause of insomnia cannot be easily made out, it 
is well to fall back upon faulty methods of training. Not 
very long since a child of five years was brought to me by 
its mother, who stated that it would not fall asleep if it 
awoke after midnight. The mother claimed to be ignorant 
of any mistake that she had made in the training of the 
child, but on closer questioning I heard that she had been 
in the habit, whenever her husband was away, of taking the 
child out of its bed and putting it in her own bed. After 
she had done this a number of times the child woke up 
regularly at midnight and would cry until the mother took 
it up. The mother, a very nervous woman, excited the 
child by her caresses and her despair over its not sleeping, 
and thus made matters worse and worse. The child had 
been sleeping very poorly for several weeks before I was 
consulted. The cure was a very simple one. The child 
was placed under the care of a sensible trained nurse, who 
would not yield to its solicitations, and after a few nights 
of restless sleep it began to develop proper habits and soon 
slept an average of ten hours. 

The prognosis is invariably favorable unless the condi- 



1 88 THE NERVOUS DISEASES OF CHILDREN. 

tion is due to some grave disorder. It is most important 
to insist upon absolute regularity in putting the child to 
bed at a definite hour and keeping it there unless it is neces- 
sary to take it up. If this should prove insufficient a warm 
bath at bedtime will be conducive to sleep, and small doses 
of bromide or chloral will be useful to engender the habit. 
Sulfonal or trional, in five- to ten-grain doses, veronal in 
three-grain doses, may be substituted for the bromide or 
chloral in children who are anaemic or poorly nourished. 
All hypnotics should be withdrawn as soon as possible, for 
there is great danger of accustoming the child to these 
drugs. In some cases the mere regulation of the meals, and 
the avoidance of all indigestible articles of diet, will be suffi- 
cient to restore sleep. In children who are impressionable 
it is of the greatest importance to avoid telling them stories 
or even engaging them in play in the latter part of the day. 

Pavor Nocturnus, or night fear, is a condition which 
is often troublesome both to the parents and the physician. 
Children affected with this disturbance wake up a few 
hours after they have entered into a sound sleep, are pos- 
sessed as a rule by great fear, fail to make out their sur- 
roundings, and act as though in a temporarily dazed con- 
dition. They do not understand the soothing words of 
parent or physician, and often continue in a state of excite- 
ment until they return to full consciousness or are over- 
come by sleep. 

There seems to be no good reason to give any other in- 
terpretation of this condition than that the child has had a 
horrible dream, and that the substance of the dream is con- 
tinued in the half-waking state, or that the child is possessed 
by fear, which is the natural result of the dream and can- 
not shake off the fear until it fully realizes, if old enough, 
that its experiences have been unreal. 

This condition may occur in any child, but it is decidedly 
more frequent in children who have shown previous nervous 
symptoms, or in children of highly neurotic parents. It is 
not infrequent in those who are pressed by school duties, 
and who go to bed with a fear that their lessons have not 
been properly done for the next day. This knowledge is 
sufficient to prevent sound sleep, and during the hours of 



THE DISORDERS OF SLEEP. 1»9 

restlessness vivid dreams are very apt to occur. It was 
formerly supposed that late and indigestible meals were 
the chief cause of these noctural disturbances. Inasmuch 
as any indigestion may give rise to restless sleep, this factor 
may have to be taken into account ; but over-work, over- 
excitement, the reading of horrible or fascinating tales just 
before bedtime, constitute a much more important etiologi- 
cal factor. 

These noctural attacks often occur a single time, but 
with most children are frequently repeated during the ear- 
lier years of life, say between the ages of three and eight 
years. With the growth of the child and the improvement 
of its nervous system the attacks cease. 

The prognosis of this condition is entirely favorable ; 
though I can recall one case of a girl, aged six years, the child 
of an hysterical mother, in whom the fright connected with 
one of these nocturnal attacks was the cause, or at least the 
first beginning, of a severe chorea, which lasted for many 
months. In another child, about nine years of age, who had 
had attacks of epilepsy ever since early childhood, and who 
was particularly subject to nocturnal attacks of epilepsy ; 
the epileptic attacks ceased upon bromide treatment, but 
every now and then the child would have a very marked 
spell of pavor nocturnus, which would disturb her almost 
as much as an epileptic seizure would. 

Conditions similar to those of pavor nocturnus occur at 
times during the day. A little patient of mine, aged five 
years, Avould, in a very unaccountable manner, while walk- 
ing with her mother on the street, suddenly hide her face in 
the folds of the mother's dress, and bury her head for some 
time, in order (as she explained later on), not to see the hor- 
rible black things coming toward her. This child showed 
no other symptom of a nervous disposition, but was for a 
time in great dread of the recurrence of these experiences. 
That they were the result of visual hallucinations during 
the waking hours, I can hardly doubt. They disappeared 
entirely under sedative treatment, which was given carefully 
for some months. 

In regard to treatment, the most important point is to ex- 
ercise every possible precaution in order that unnecessary 



I90 THE NERVOUS DISEASES OF CHILDREN. 

nervous excitement, late in the day, may be avoided I 
prohibit all school-work after five in the evening ; do not 
allow the child to read or be read to after this hour, and in- 
sist on a very light meal in the evening. After this meal 
the child is to remain awake for at least an hour, and may 
indulge in some simple game, or is allowed to frolic about 
in a quiet manner. Small doses of the bromides, from five 
to ten grains, according to the age of the child, about an 
hour before bedtime will be the best remedy. If neces- 
sary, small doses of chloral may be given with the bromides. 
If the attacks return very frequently I have found it more 
satisfactory to give five- or ten-grain doses of chloralamid, 
or of trional, every night, or every second or third night, 
until all excitement has subsided. 

Enuresis Nocturna is another very common form of 
disturbed sleep. Children who have been well trained, 
and who have learned to observe all the habits of cleanli- 
ness during the day, are frequently disturbed by involuntary 
micturition during the night. This is most apt to occur 
during the earlier hours of sleep, when sleep is most pro- 
found. The soundness of sleep alone may be a sufficient 
cause for this phenomenon, inasmuch as children do not 
perceive in sleep those symptoms of vesical irritation which 
prompt them to evacuate the bladder during waking hours, 
and during light sleep. In others, and possibly in the ma- 
jority of cases, it is due to indifference on the part of the 
child, or to an aversion to allow its sleep to be interrupted. 
In some few cases enuresis is the result of frightful dreams, 
the sudden impulse to evacuate the bladder accompanying 
such a dream as it would any emotional condition during 
wakefulness. If due to this latter cause the enuresis is not 
frequently repeated. Enuresis has been observed to be a 
symptom of nocturnal epilepsy. If so, it occurs at rare in- 
tervals ; the child is entirely unconscious of it, and often 
shows some other symptom, such as drowsiness and head- 
ache on the following morning. 

The condition is a very stubborn one, and all possible 
methods of treatment have been suggested. I am con- 
vinced that the cure of the condition is dependent chiefly 
upon careful training. The necessity of evacuating the 



THE DISORDERS OF SLEEP. I9I 

bladder should be impressed upon children after the age 
of two years. The child should be made to void urine im- 
mediately before going to bed, and should be aroused one 
and a half to two hours after it has fallen asleep, and should 
be induced to pass water again. By giving relatively few 
liquids after four or five o'clock in the afternoon, I have 
found that the tendency to enuresis is often checked. 

If these simple measures are not sufficient, a few drops 
of the tincture of belladonna, given at bedtime, will be use- 
ful, and if the condition is due to frightful dreams, or to 
cerebral excitement continued during sleep, small doses of 
bromides may be given. Many medical men have seriously 
suggested that corporal punishment applied to the nates is 
the only efficient remedy, and some have gone so far as to 
suggest that the cutaneous hyperemia caused by such pun- 
ishment explains the relief afforded. This explanation is 
not satisfactory ; but whatever the mode of action may be, 
it is very certain that such punishment need not be inflicted 
in the majority of cases, and that the desired end can be 
attained by other methods of training. 

Somnambulism is by no means rare in children, and occurs most fre- 
quently in those who are of a nervous temperament, or who have passed 
through some severe excitement. I have not known them to perform 
any of the marvellous tricks which are generally accredited to somnambulists, 
such as climbing out of windows and on the roof, and the like ; but they are 
apt enough to walk through the house, from one room to another, and to go 
through a number of purposive actions without being at all conscious of what 
they are doing. Nightmare is evidently closely related to this condition of 
somnambulism, and is generally associated with horrible dreams, from 
which the child awakens badly frightened, but is quieted much more rap- 
idly than in the condition which is described as pavor nocturnus. Night- 
mare and somnambulism occur, as a rule, at rare intervals and can generally 
be traced to some emotional excitement, or to some acute indigestion. The 
treatment of these conditions is exactly the same as that of night-terror. 



CHAPTER XL 

VASOMOTOR AND TROPHO-NEUROSES. 

In this chapter a brief description will be given of a num- 
ber of rare and peculiar diseases, which must for the pres- 
ent be classified under the rather vague title of vasomotor 
and tropho-neuroses. Grouping them together under one 
head is simply a matter of convenience. 

EXOPHTHALMIC GOITRE. 

This disease, which is generally termed Graves's disease 
by the English writers, and Basedow's disease by the Ger- 
mans, occurs chiefly in adult life, yet a sufficient number of 
cases begin during childhood to make it incumbent upon 
us to give a brief description of the disorder. 

The cardinal symptoms are : 

i. Excessive cardiac action. This is by far the most 
constant symptom of all, and is often present in the earlier 
stages of the disease when the other two cardinal symptoms 
— swelling of the thyroid and the protrusion of the eyes — 
are not yet in evidence. The pulse-rate may vary between 
90 and 200. In the later stages of the disease the left ventri- 
cle, and rarely the right ventricle, may become hypertro- 
phied and dilated. The heart-sounds are clear but unusu- 
ally loud. All the arteries pulsate very distinctly, so that 
the pulse of the abdominal aorta may possibly be observed 
through the abdominal walls, and the pulse in the carotids 
may be so strong as to cause distinct pulse-like vibrations 
of the entire head. The pulse may even be noticeable in 
the retinal arteries, and a further disturbance of the entire 
vascular system may be noted by a dilatation of the capil- 
laries in the skin, by the presence of distinct tdches cere- 
brales, and a dilatation of all the larger superficial veins. If 



VASOMOTOR AND TROPHO-NEUROSES. 193 

venous stasis is marked, oedema may occur in various parts 
of the body. It is also owing to the increased tension that 
hemorrhages are frequent from the nose, and even in the 
stomach and bowels. Cerebral hemorrhages have also been 
described in Graves's disease. A particularly characteristic 
symptom, which is found in exophthalmic goitre, and not 
in any form of enlargement of the thyroid, is the whirring 
felt if the finger is placed upon the goitre. 

2. The second cardinal symptom is the enlargement of 
the thyroid gland. It is present in the vast majority of 
the cases, though a number of authors have taken pains to 
describe cases of this vasomotor disease in which the 
thyroid was not enlarged ; but the enlargement, as a rule, 
comes on some time after the tachycardia. The gland, after 
it has once become enlarged, increases rapidly, and may give 
rise to a great deal of inconvenience. The enlarged gland, 
or struma, is extremely vascular, and the tissue of the gland 
is not only hyperaemic but very soon enters into a -state of 
hyperplasia with a tendency to a fibrinous degeneration. 
In keeping with these changes the goitre may in the begin- 
ning be soft and yielding, later on it will become hard to the 
touch. The enlargement of the thyroid is, as a rule, bilateral, 
but generally asymmetrical. The right half is more fre- 
quently enlarged than the left half. 

3. The third cardinal symptom is exophthalmus, or pro- 
trusion of the eyes. This is the least constant of the three 
symptoms. If present it is generally bilateral, but in some 
instances one eye only may be affected, or the protrusion of 
one may be very much greater than that of the other eye. 
The causes of this protrusion have been in dispute, but it is 
more than probable that the interference with the venous 
current, as well as the arterial congestion, are the prime 
causes of this protrusion. The great variability of this 
symptom during life, its disappearance almost immediately 
after death, and the pulsation of the vessels in the fundus, 
lend support to this view. It can hardly be doubted, how- 
ever, that in some cases the development of fat and of cellu- 
lar tissue in the retro-bulbar space may make the protrusion 
still more marked. 

Vision is not affected in spite of the exophthalmus, and 



194 THE NERVOUS DISEASES OF CHILDREN. 

the only changes observed were those recorded by Kast 
and Willbrand, who have noted a limitation of the field of 
vision in this disease without any evidence of hysteria. The 
pupils may be unequal, but react promptly. Ulcerations of 
the cornea, occurring in consequence of insufficient protec- 
tion of the bulb, have been noted ; as well as some interfer- 
ence with the lachrymal secretion. 

Among the accessory symptoms those relating to the 
movements of the eyes are the most important. Graefe's 
symptom refers to defective movements of the upper eye- 
lid on vision downward. The upper lid does not follow 
promptly the downward movement of the bulb, and the con- 
junctiva remains visible between the pupil and the margin 
of the lid. This symptom evidently bears some relation to 
the phenomenon described by Stellwag consisting of a dila- 
tation of the palpebral fissure. Graefe's symptom, as far as 
can be ascertained, is caused neither by the protrusion of 
the eyes nor by a spasm of the levator or a paresis of the 
orbicularis, but is explained most readily as the result of 
Stellwag's phenomenon. Moebius has described another 
symptom implying a deficiency in the power of accommo- 
dation. There is defective convergence of the axes of the 
eyes. These three symptoms are not absolutely pathogno- 
monic of Basedow's disease, as each one has been described 
in connection with other neuroses; but if they are associated 
with either one of the cardinal symptoms they help to cor- 
roborate the diagnosis of Basedow's disease. 

The next most frequent, and perhaps most important, 
symptom is a tendency to profuse diarrhoea. This is en- 
tirely independent of any gastro-intestinal disease, often 
resists treatment, and adds greatly to the patient's discom- 
fort and to the general depreciation of his health. Gastric 
crises have been described in some cases, and frequent 
vomiting is not an unusual symptom. Most patients exhibit 
considerable dyspnoea, resembling true asthma, and Louise 
Bryson has referred to defective expansion of the chest on 
inspiration. Glycosuria and albuminuria have been re- 
ported ; the former, according to the investigations of Chvo- 
stek, is of the alimentary order. In some few instances, 
however, diabetes mellitus has occurred as a complication 



VASOMOTOR AND TROPHO-NEUROSES. 1 95 

of Basedow's disease. This association is not a strange one, 
since both diseases are often developed after severe emo- 
tional excitement. Of the disturbances in menstruation we 
need take no account, as we are, after all, concerned with 
the disease as it occurs in children. 

Trophic and vasomotor disturbances of the skin are fre- 
quent. Pigmentation, leukoderma, loss of hair, premature 
grayness of hair, and even scleroderma, as well as herpes, 
have been observed. A very remarkable symptom of 
Graves's disease is a tendency to profuse sweating. This 
may be universal or partial, sometimes strictly unilateral. 
This tendency must be held to account for the remarkable 
diminution in electrical resistance which has been observed 
in the skin of patients suffering from this disease. This fact 
was first insisted upon by Vigouroux, and later by Eulen- 
burg. Kahler and others have shown that this reduction in 
resistance to the galvanic current is due to the fact that the 
relative minimum of resistance is reached much more easily 
in patients suffering from Graves's disease than in healthy 
individuals. Kahler has also shown that this diminished 
resistance is not due to an hypersemia, as was supposed by 
some, but it is due to excessive moisture of the skin. A 
faint tremor is frequently observed, and by some is con- 
sidered to be one of the cardinal symptoms, but it is not as 
constant as the three cardinal symptoms we have mentioned. 
The tremor is rhythmical and consists of eight or nine vibra- 
tions per second. Choreiform tremor occasionally occurs, 
and true chorea and epilepsy are complications met with 
in a certain number of cases. These complications are in 
all probability purely accidental, and cannot readily be ex- 
plained on the supposition that the vasomotor changes in 
Basedow's disease are the cause of the chorea and epi- 
lepsy. 

In addition to the preceding symptoms mental changes 
deserve some notice, and among these we may note irrita- 
bility of temper, sudden changes of temperament, and the 
occasional occurrence of mania or melancholy. Some of 
these psychic changes are developed in the earlier stages of 
the disease, others are later, and possibly accidental compli- 
cations. If we add anaemia, albuminuria, and a general 



196 THE NERVOUS DISEASES OF CHILDREN. 

cachexia, we have completed the list of the symptoms typi- 
cal of Graves's disease. 

The morbid anatomy of Graves's disease has been the 
subject of much discussion. The cervical sympathetic has 
been given a very important part. A number of authors 
have reported actual changes in the cervical ganglia as the 
result of connective-tissue proliferation, and others have re- 
ported atrophy and degeneration of the nerve-elements ; but 
since Hale White has proven that similar changes occur in 
persons who have not exhibited the symptoms of Graves's 
disease, and that in persons who have died from this disease 
such changes have not been found, the force of all these 
findings has been much diminished. The same may be said 
of hemorrhages into the fourth ventricle, or atrophy of the 
corpus restiforme, to which Mendel and Leube have attached 
some importance. The heart has naturally exhibited hyper- 
trophy or dilatation, insufficiency of the mitral valve, and 
symptoms of mild endocarditis, but these changes are 
clearly secondary and do not at least hold any causal rela- 
tion to the disease. 

The theories regarding the pathology of the disease 
have been still more conflicting. Basedow supposed the 
disease due to chlorotic changes in the blood ; but this ex- 
planation is evidently insufficient, as the disease frequently 
affects persons in robust health who are far from anaemic. 
The close resemblance between the symptoms following 
upon section of the cervical sympathetic and those of 
Graves's disease has led many writers to attribute exophthal- 
mic goitre to disease of the sympathetic ! Section of the 
sympathetic produces irritation, and the symptoms follow- 
ing the experiment upon animals are clearly the result of 
irritation ; but we cannot suppose any condition of irritation 
to last for years, and must therefore seek some other expla- 
nation. Friedreich was inclined to the theory that dilatation 
of the vessels is the result of paralysis of the sympathetic; 
that dilatation of the coronary arteries caused tachycardia, 
and that this increased flow of blood to the cardiac muscle 
produced an increased activity of the heart, and that all the 
other symptoms of Graves's disease, the exophthalmus and 
the thyroid swelling, were the result of the arterial hyper- 



VASOMOTOR AND TROPHO-NEUROSES. 1 97 

aemia. Granting- that the cardinal symptoms can be ex- 
plained in this way, the many accessory symptoms cannot 
be accounted for on this theory. Other authors have in- 
sisted on the bulbar origin of Graves's disease, and have sup- 
posed that a lesion involving the nuclei of the vagus was 
sufficient to account for all the symptoms, and some post- 
mortem findings have given support to this view. 

A number of authors, more especially Moebius, have in- 
sisted on the probability of the toxic origin of the disease, and 
have supposed that by the increased action of the thyroid 
gland a toxine is produced which is primarily responsible 
for all the symptoms. In a recent and very able review of all 
the conflicting theories (hematogenic, neurogenic and thyre- 
ogenic), Eulenburg (Die Deutsche Klinik, 1904) concludes 
that there must be qualitative as well as quantitative 
changes in the secretion of the thyroid gland to account for 
the symptoms of Basedow's disease. These quantitative 
changes, Eulenburg supposes, are due to an abnormal nerve 
influence, the exact nature of which is still to be determined. 

The course of the disease is, as a rule, extremely chronic. 
The onset may be very sudden, and even violent, but after a 
while the symptoms subside in severity and remain con- 
stant for a long period of years ; but the prognosis is not 
necessarily as grave as it is generally supposed to be. I 
have myself seen a number of cases of very marked im- 
provement and of total disappearance of all the symptoms. 
If death ensues it is, as a rule, due to some complicating 
disease, or to extreme exhaustion from diarrhoea, or from 
cardiac weakness, or possibly from acute mania. 

Treatment. — Absolute rest and careful feeding are the 
most important measures. The recoveries which I have 
seen have been in patients who have been placed on the 
rest-cure. The entire freedom from excitement, and regular 
feeding have tended to lessen all the nervous symptoms and 
have influenced the diarrhoeas which are often the most ex- 
asperating and exhausting symptom. The tachycardia is 
remarkably lessened and the dyspnoea is naturally benefited 
by rest in bed. The diet should be simple. It is well to 
avoid stimulants and excitants as well as carbonated waters. 
The excessive dilatation of the stomach, resulting from the 



I98 THE NERVOUS DISEASES OF CHILDREN. 

last-named, acts unfavorably upon the heart. Hydrothera- 
peutic procedures, consisting of cold baths followed by 
massage, of douches and drip sheets, have been recom- 
mended by many. Mineral baths may have some effect 
upon the constitutional symptoms and thus favorably in- 
fluence the disease. Electricity has been warmly recom- 
mended by many, with special reference to the part played 
by the cervical sympathetic and vagus. To give this 
method a fair trial it is best to place the anode by turns 
over the goitre, the heart, and over the abdomen. The cur- 
rent should be mild and continuous. Recently Rockwell 
has recommended the use of the faradic current. 

As far as medicinal treatment is concerned the drugs in- 
fluencing the heart's action have been most in favor ; above 
all, digitalis and strophanthus. While these drugs act 
favorably in some cases they unquestionably exert an un- 
favorable influence in others. I have found that exces- 
sive palpitation of the heart could be controlled very much 
better by cold applications to the cardiac region, and by 
the use of the galvanic current, with the anode over the 
heart. I am willing, however, to concede that the effect of 
the latter may be due to suggestion. If there is great 
emotional excitement bromides in small doses may be given 
with advantage. Nitrite of amyl and nitro-glycerine have 
been recommended, but they are to be used with great cau- 
tion, especially in children, and the result is a doubtful one 
at best. Ergot has been administered in some instances, 
and the subcutaneous injections of ergotin into the goitre 
have been practised, but the results have not been favorable 
enough to warrant its continuance. 

Thyroidectomy, or excision of a part of the enlarged 
gland, has come into vogue, and has been reported by 
many German, English, and American writers. Improve- 
ment following the operation has been observed, and in my 
opinion is sufficiently marked to warrant further trial of 
this procedure ; but I am not aware that the operation has, 
as yet, been attempted in children. Sufficient time has not 
yet elapsed to decide whether the relief obtained by the 
operation is merely temporary, or whether a permanent 
cure has been effected. If the enlarged gland presses upon 



VASOMOTOR AND TROPHO-NEUROSES. 1 99 

the trachea and menaces life, the operation is surely justi- 
fied. Moebius, Leyden, and others have advised the use of 
Anti-thyroidin, Rodagen (prepared from the milk of goats 
whose thryoid glands had been removed) ; but the effect of 
these newer remedies is still sub judice — and great caution 
should be exercised in administering them. 

Thyroid Enlargements at the Age of Puberty. — At this period 
the enlargement is often developed quite suddenly, and is so marked as to 
arouse suspicion of incipient exophthalmic goitre. As such girls are often 
anaemic, and have a high pulse-rate the suspicion of serious disease becomes 
still stronger ; but such enlargement of the thyroid gland does not continue 
for any great length of time, and none of the other important symptoms of 
Graves's disease are developed. 

Tachycardia should also be mentioned, as it may occasionally give rise 
to the suspicion of Graves's disease. An excessively high pulse-rate is fre- 
quent in children, but true tachycardia in children has, to my knowledge, 
rarely been reported. I have seen several interesting cases of this kind, and 
one of them was of unusual interest. It was the case of a boy, of twelve 
years of age, who had been under my treatment for infantile cerebral hemi- 
plegia, and had sufficiently recovered from this condition to be able to go 
about freely, to attend school, and to play with boys of his age. While 
playing on a cold winter's day he was struck in the neck by an icy snow- 
ball. Directly he felt faint, but did not lose consciousness. As soon as he 
was put to bed the tachycardia became evident, and his family physician was 
sent for. From the first day until about three weeks after the accident the 
pulse was at no time less than 200 per minute, and often exceeded this rate, 
so that the number could not well be counted. None of the drugs ex- 
hibited—such as bromides, strophanthus, digitalis, aconitia — had the slightest 
effect upon the heart ; but after a period of about three weeks the symptoms 
subsided quite suddenly, and the boy, now a man, has been entirely well for 
many years. The injury to the cervical region, and possibly to the cervical 
sympathetic, causing tachycardia, is of interest, if we recall the part that the 
cervical sympathetic is supposed by many to play in the causation of Graves's 
disease. The treatment of such cases should consist of rest, and the appli- 
cation of an ice-bag to the heart and to the cervical spine ; bromides, heart 
tonics, and aconitia may be tried according to the indications of the case. 

MYXCEDEMA. 

Myxcedema is a form of trophic disease to which attention should be 
directed. The disease is of unusual interest, but we need not go into a full 
discussion of the subject, as the one form of it which occurs in children — 
myxcedematous idiocy — will be referred to in the Chapter on Idiocy. Myx- 
cedema was first fully described by Gull, in 1873; four years later similar 
cases were reported by Ord, and since that time innumerable authors in 



200 



THE NERVOUS DISEASES OF CHILDREN. 



England, America, France, and Germany have studied this trophoneurosis. 
Special interest has been attached to this disease, since Kocher described a 
condition following upon removal of the thyroid, resembling myxcedema, to 
which he gave the name, cachexia strumipriva. The Commission that re- 
ported to the Clinical Society of London, in 1888, agreed that myxcedema, 
cachexia strumipriva, sporadic cretinism, represented one and the same mor- 
bid entity. The condition following upon removal of the thyroid gland, 

the results of physiological ex- 
periments (implantation of the 
glands into the abdomen of 
animals — Schiff, Horsley, and 
others), and above all, the brill- 
iant achievements of thyroid 
feeding, have proved conclu- 
sively that deficient action of 
the thyroid gland is the most 
important factor in the etiology 
of myxcedema. 

Symptoms. — Of the symp- 
toms of myxcedematous idiocy 
(the congenital form) we need 
here merely state that the skin 
is glossy or hard ; the lips and 
tongue are thick and large ; the 
hair is stiff and dry ; the child 
is dwarfish in stature ; the men- 
tal condition is that of marked 
idiocy or of imbecility. There 
is always absence of the thyroid 
gland. 

Myxcedema resembling that 
of the adult may come on at 
the age of puberty, or even ear- 
lier. It is characterized by the 
following symptoms : A gen- 
eral increase in the bulk of the 
body ; the skin is firm and in- 
elastic, does not pit upon press- 
ure, and is dry and rough ; the 
folds of the skin disappear, and there is a general obliteration of all the lines 
of the skin, particularly in the face, giving, as a rule, an older and more stu- 
pid expression to the face ; the nostrils and lips are very much enlarged. 
There is distinct apathy and slowness of speech, as well as of action, in some 
instances ; the mental changes include delusions which lead to dementia. 
Albuminuria and glycosuria have been observed ; but these are accidental 
complications, no doubt. 

The disease cannot be mistaken for any other, except possibly chronic 




Fig. 52. — Case of Myxoedema with Idiocy. Pa- 
tient Twelve Years Old : Dwarfish in Stature. 



VASOMOTOR AND TROPHO-NEUROSES. 201 

nephritis ; but the lack of pitting, the examination of the urine, and the pe- 
culiar expression of the face will help to remove all doubts. 

The disease is slowly progressive, and may last for ten, fifteen, or more 
years. The prognosis was hopeless until the recent discovery of thyroid 
feeding, and success of this warrants us in rejecting for the present every other 
form of treatment. 

Treatment is to consist of the administration of the pulverized thyroid 
gland of the sheep, which is to be had in all countries. Armour's prepara- 
tion has, in my experience, been the most satisfactory. Patients taking thy- 
roid gland should be observed carefully. 

In children one grain twice a day should be the maximum dose at the be- 
ginning ; this may be increased to three or five grains twice daily until the 
amount is established which the patient needs to keep in a normal condition. 

A word of caution is in order not to continue the remedy if muscular pain 
and cardiac attacks occur. In several cases I have been compelled to discon- 
tinue the use of the gland on account of extreme and rapid emaciation. 
Tonic measures should be employed promptly if the general health of the child 
has been impaired. 

ANGIONEUROTIC (EDEMA. 

Angio - Neurotic CEdema is a trophic disorder, first described by 
Quincke, in 1882, although conditions closely resembling, or identical with it, 
have been known ever since 1827. It has passed under the designations of 
" acute circumscribed oedema," " periodic swelling," " Australian blight," 
"non-inflammatory oedema," etc. 

The disease is characterized by the appearance of circumscribed swell- 
ings in various parts of the body, more especially in the face, throat, and the 
extremities. These swellings appear without any direct cause, and are often 
associated with gastro-intestinal disturbance, which is thought to be due to 
a condition of the mucous membrane of the stomach and bowels similar to 
that of the skin or larynx when these parts are diseased. The disease comes 
on in attacks, which are precipitated most easily by exposure to cold, and by 
slight or severe injuries. The attacks are most apt to occur between the 
hours of 1 and 5 a.m. In 71 cases the parts first affected were : The face in 
29 cases ; the extremities in 22 cases ; the larynx in 5 cases ; the genitals 
— penis, scrotum — in 3 cases ; the trunk in 6 cases ; the stomach in 3 cases; 
the gums, neck, ear, each in 1 case. 

The oedema may be fully developed within one-half to two hours. The 
color of the skin is either whitish, or a dull roseate hue with a whitish shad- 
ing near the centre of the cedematous patch. There is no pitting on pressure, 
and none of the signs of an inflammatory swelling. The swelling, if in the 
face, may be marked enough to cause disfigurement. The sudden jumping 
of the swelling from one part of the body to another is quite characteristic. 
Subjectively there is a sensation of scalding or burning, or an itching on the 
part affected ; and after the oedema subsides a " heavy wooden " feeling is 
complained of. The disease is not a serious one unless it affects the mucous 



202 THE NERVOUS DISEASES OF CHILDREN 1 . 

membranes of the larynx and pharynx ; if the oedematous swelling in the larynx 
increases rapidly, death may take place from asphyxia, as in two cases re- 
ported by Osier. One case has been reported that proved fatal from oedema 
of the glottis (Krieger). 

The gastro-intestinal tract, if affected, may exhibit severe disturbance, 
such as pain in the epigastrium, vomiting, colic, retraction of the abdomen, 
and constipation or diarrhoea. 

(Edema of the lungs has been reported as a symptom of this disease ; but 
proof of this is wanting. 

Collins has analyzed 75 cases. 

According to this author the average age at the onset of the disease is 
twenty-seven ; childhood is by no means exempt. Dinkelacker has reported 
the case of a child that had its first attack when three months old ; similar 
cases have been reported by Widonitz and Goltz. The disease occurs nearly 
twice as often in males as in females. It may come on after any exhausting 
condition ; but heredity, as well illustrated in a family described by Osier, is 
the most important factor. In this family five successive generations have 
been affected. Severe emotional excitement and hysterical conditions are 
the forerunners of the disease. The ingestion of certain foods was followed 
by this special form of oedema in cases reported by Osier, showing an etio- 
logical resemblance at least between angio-neurotic oedema and urticaria. 

Matas and others have described cases of this disease coming on after 
or during malarial fever. The diagnosis is made readily enough ; it may be 
confounded with the blue oedema of hysteria, as described by Sydenham, or the 
white oedematous swellings of hysteria described by Charcot ; but the pres- 
ence or absence of the stigmata of hysteria will help to disprove or corrobo- 
rate the suspicion of angio-neurotic oedema. 

There can be little doubt that angio-neurotic oedema is a vasomotor 
neurosis ; it is analogous to the non-inflammatory swellings which Weir Mitch- 
ell described after injury to the peripheral nerves, and to similar swellings, 
which appear after stretching a nerve. I have seen oedematous swellings in 
the face, after section of the trigeminal nerve, very like the circumscribed 
oedema under discussion. The serous exudation in all probability results 
from a retardation of the blood-current, and this in turn must be attributed 
to a local paresis of the vaso-constrictors, or a reflex stimulation of the vaso- 
dilators. 

Treatment of this disease as it occurs in children is more difficult than in 
the adult, in whom the removal of toxic causes, such as alcohol and tobacco, 
is all that is needed. In children the hereditary element is most marked, 
and all we can hope to do is to inhibit the attack and to strengthen the 
general nervous system. To check the attack we may compress the affected 
part by an elastic bandage, or apply dry heat. Small doses of morphia or 
codeia may be given. In the case of swelling in the pharynx or larynx, sur- 
gical interference may be necessary. To improve the general health of the 
child it will be best to administer strychnia in small doses (grain one-hun- 
dredth to grain one-sixtieth), and to give blood-tonics such as iron and ar- 
senic. 



VASOMOTOR AND TROPHO-NEUROSES. 203 



RAYNAUD'S DISEASE. 

Raynaud's Disease, or symmetrical gangrene, is a very rare tropho- 
neurosis, which occurs, however, quite as frequently in children as in adults. 
Indeed, says Morgan, " if there be any period when it is especially prone to 
occur, it is in childhood." Of 93 cases which he analyzed 24 were in children 
under ten years of age, and 5 of Raynaud's original cases were children be- 
tween three and nine years old. Mendel observed a case in a child fifteen 
months old r 

The symptoms of the disease are practically the same as those first enu- 
merated by Raynaud in 1862. A localized ischasmiaor asphyxia in symmetri- 
cally situated parts is the most constant symptom ; this asphyxia may lead to 
gangrene, but does not invariably do so ; nor is the disease invariably sym- 
metrical, and for that reason the designation of symmetrical gangrene is not 
entirely appropriate. The order of development of the symptoms may vary 
somewhat. Before the local syncope is fully developed, there is often a 
general numbness and some pain in the part to be affected. The affected 
area becomes pale and waxy in appearance ; if pricked, little or no blood flows 
from it. This condition may be recovered from, and several such attacks 
may occur, each one ending in recovery ; finally, however, the condition per- 
sists, and local asphyxia is developed ; in some instances the condition is 
reached at once and is persistent. The affected areas become deep red, then 
blue, and finally black in color ; the parts are swollen, and the local temper- 
ature is lowered. Extravasation of blood into the surrounding tissues may 
occur. Recovery at this stage is still possible ; but if the morbid process is 
continued for some time the parts grow thinner, the fingers become atten- 
uated, and finally gangrene results. The gangrene comes on either some 
months after the first symptoms of local asphyxia, or it is developed at the 
earliest period of the disease. The tips of the fingers and toes may be de- 
stroyed by this process, or an entire hand or foot or some other considerable 
part may be destroyed by gangrene. The parts most frequently affected, 
are the fingers, toes, ears, the buttocks, the calves, and the nose. The gan- 
grenous area is generally separated from healthy tissue by a sharp line of 
demarcation ; the dead part may either become mummified, or it may be 
cast off after suppuration. Moist gangrene also occurs at times, with the for- 
mation of large bullae and pus centres. If a gangrenous area heals, it behaves 
like a torpid ulcer (Elliot). 

Intermittent haemoglobinuria and glycosuria have been observed. The 
pathology of the disease is still obscure. Raynaud and his successors were 
of the opinion that the local syncope is produced by a contraction or spasm 
of the blood-vessels (both arteries and veins). The disease of the blood- 
vessels is by several authors attributed to syphilis or Bright 's disease. The 
symptoms of Raynaud's disease have also been observed in association 
with tabes, syringomyelia, myelitis, and neuritis. 

The prognosis is generally good ; if death occurs, it is due to some in- 
tercurrent disease. Recoveries are frequent, even if local gangrene has 



204 THE NERVOUS DISEASES OF CHILDREN. 

existed ; but if large portions become gangrenous, the general health of the 
patient may be seriously impaired. In such cases tuberculosis may be de- 
veloped and hasten death. 

Treatment should be directed toward the improvement of the patient's 
general condition, and freedom from all emotional excitement should be in- 
sisted upon. The affected parts should be wrapped in cotton, and placed in 
a position most favorable for the circulation. Dry heat or warm baths may 
be employed to sustain the temperature of diseased areas. Nitro-glycerine 
and nitrite of amyl have been recommended, but they have not been exhib- 
ited successfully. Electric (galvanic) baths, and the application of the gal- 
vanic current through the cervical spine and the affected area have also 
been favored by some ; but very little good has resulted from such treatment. 
The greatest benefit will be derived from surgical measures after gangrene 
has set in. 

FACIAL HEMIATROPHY. 

Facial Hemiatrophy is a rare form of disease. Often it begins in 
childhood, and has important relations to other diseases of the nervous sys- 
tem. It has been described by some with exophthalmic goitre and with mi- 
graine, but its relations appear to me to be very much closer to scleroderma, 
which occasionally precedes it, and I have recently seen a case of universal 
scleroderma in which there was double hemiatrophy of the face. 

The disease is characterized by a distinct diminution in the size and bulk 
of the subcutaneous tissues, and by the alteration in the bony structure as 
well. In one case, on the left side the distance from the middle of the chin to 
the angle of the jaw was 1 1 ctm. ; on the right side, \iyi ctm. ; from the upper 
margin of the naso-labial fold to the middle of the ear was 10^ ctm., while 
on the right side this distance measured \\ x /z ctm. The first indications of 
atrophy are found in the skin. A single spot, generally in the cheek, be- 
comes thin and white from the disappearance of pigment. The loss of fatty 
tissue underneath causes a depression also at this point. The atrophy pro- 
ceeds from this and gradually involves the entire half of the face. The eye 
is retracted, there is distinct wasting of one-half of the nose, and even the 
ear of one side may be smaller than that of the other side. In some few in- 
stances a slight glossiness of the skin has been observed, but in the majority 
of cases there is unusual dryness, the condition resembling scleroderma. 
The hair also is apt to be stiff and hard. In some instances the teeth in 
the affected half are small, and undergo decay. There is no disturbance 
of sensation, and no interference with any of the special senses in the ordi- 
nary run of cases. The temperature sometimes varies a little. In a case 
which I reported the temperature was one degree higher in the normal ear 
than in the ear of the affected side. The muscular movements, in spite of 
the increasing atrophy, as a rule, remain normal. Several other authors, 
and myself, have reported cases in which there were distinct tonic and 
clonic contractions of the muscles supplied by the fifth nerve of the affected 
side. Atrophy of one-half of the tongue also occurs in some cases, but it is 
a rare complication. 



VASOMOTOR AND TROPHO-NEUROSES. 205 

The causes of the disease are unknown, except that several cases have 
been developed after severe injuries to the face. Thus in one case re- 
ported by Skryme, and quoted by Gowers, in a child three and a half years 
of age, the disease was developed six months after an accident in w T hich " the 
face was jammed and the neck twisted. " The disease has also been devel- 
oped after acute infectious diseases, and after other causes which give rise to 
neuritis. There can be no doubt, however, that in many cases the disposi- 
tion to the disease is congenital. 

Pathology. — There was much dispute regarding the origin of this dis- 
ease, some claiming that the cervical sympathetic was responsible, others 
making it a disease of the trigeminal nerve. There is no doubt that a con- 
dition very similar to hemiatrophy will follow upon lesions of the cervical 
sympathetic ; but an autopsy performed by Mendel on a case which had 
been observed by many other men, proved beyond a doubt that in that case, 
at least, the disease was due to a proliferating interstitial neuritis of the left 
fifth nerve, and that this neuritis was most marked in the second branch ; in 
that case the facial nerve was entirely normal, but the left musculo-spiral had 
undergone the same changes as the left trigeminal. My own case, in which 
there w T ere marked clonic and tonic contractions of the masseters, would also 
tend to show that the trigeminus was the chief seat of the trouble, and that 
in some instances the motor, as well as the trophic fibres in the trigeminus 
can be involved in the same morbid process. The occurrence of a neuritis 
of other nerves, as well as the association of facial hemiatrophy with sclero- 
derma, would seem to prove that the morbid process may in some cases ex- 
tend beyond the fifth nerve. 

The course of the disease is, as a rule, steadily progressive, though after 
the lapse of time there may be a complete standstill of all the symptoms. 
The disease does not call for any active treatment, excepting in cases like my 
own, in which the spasmodic contraction of the masseters was extremely 
painful and had to be relieved by opiates. The disfigurement of the face is 
the most serious feature of the disease. In the case of one patient I suc- 
ceeded in rounding out the cheek again by inserting a light rubber plate, 
which was attached by a dentist to the patient's upper teeth.* 

* In view of the excellent results obtained by thyroid feeding in a case of hemi- 
atrophy with scleroderma, this treatment deserves a fair trial. 



PART II. 

ORGANIC DISEASES OF THE NERVOUS 

SYSTEM. 



CHAPTER XII. 

DISEASES OF THE PERIPHERAL NERVES. 

Traumatism, toxic infections, exposure to wet and cold, 
rheumatism, the causes which lead to disease of the pe- 
ripheral nerves in the adult are not frequent in children. A 
description will be given of those forms only which are 
commonly met with in the earlier years of life. 

In diseases of the peripheral (mixed) nerves, whether 
there be an inflammatory or degenerative process, the symp- 
toms are, as a rule, motor and sensory in character. The 
paralysis is limited to the muscles supplied by the diseased 
nerve and by branches coming off below the site of injury or 
disease. The sensory symptoms may be a hyperaesthesia or 
an anaesthesia, according to the degree of change. Paraes- 
thesiae are very common, and if the nerve is inflamed it is 
painful on pressure. It is a striking fact, however, that the 
motor fibres suffer much more readily than the sensory fila- 
ments; possibly because the former are farther removed 
from the nutrient cell, and probably because a collateral in- 
nervation between sensory fibres is more easily established. 
Sensory fibres are also regenerated more easily. Further 
symptoms are, marked atrophy, loss of reflexes in the dis- 
eased member, and changes in electrical reaction. The 
degree to which the last are developed gives an indication 
of the amount of degeneration in the nerve. 

Trophic disturbances are very common in connection 
with peripheral neuritis and degeneration of peripheral 
nerves ; but these disturbances do not, as a rule, appear ex- 
cept in severe cases. Local cyanosis, oedema, and ulcers are 
common ; the skin becomes smooth and glossy, and the 
growth of the nails may be interfered with. 



210 



THE NERVOUS DISEASES OF CHILDREN. 



In children, as in adults, the nerves of the brachial plexus 
are most frequently affected. 

The symptoms referable to lesions of the brachial plexus 
vary according- to the part of the plexus involved. As in the 
adult, we can distinguish between several distinct types ; 
the upper-arm type, the lower-arm type, and the paralysis 
due to total plexus lesion. 



THE OBSTETRICAL PALSIES. 



As the name implies, these palsies are due to manipula- 
tions during labor. If the shoulder and clavicle are pressed 



jflnt.Tlicracic 



NSupra-Scap. 




NCircnmflex. 
JfAtusc.Cut 



^^ ;raI 



Fig. 53. — The Brachial Plexus : the Branches involved in the Upper Arm Type of 
Paralysis are indicated by Shading. 

backward and upward injury to the plexus may result there- 
from. In cases of breech presentation pressure upon the 
supra-clavicular spaces may cause a partial plexus paralysis. 
I have seen several cases in which this paralysis was bilat- 
eral. The disturbances that ensue upon these obstetrical 
manipulations may be the result of a simple traction upon 
or of laceration (Pierce Clark) of some or of all the fibres 
of the brachial plexus (Erb's type most frequent). 

The affected arm hangs down limp. Although the child 
moves the fingers and the arm of the normal side, there 
is little or no power on the paralyzed side. In the first 



DISEASES OF 7 "HE PERIPHERAL NERVES. 211 

few weeks after birth, if electrical examination is attempted, 
it will be found that the muscles of the affected extremity 
do not respond to the electric currents. At later periods, 
in the milder cases, the galvanic response of the nerves may 
be a little altered, but the muscles, as a rule, do not exhibit 
any marked change. If the case is a more severe one the 
diseased branches of the brachial plexus will fail to respond 
both to the galvanic and faradic current, and the muscles 
supplied by these branches will show either an altered 
galvanic response, the anodal closure contractions being 
greater than the cathodal closure contractions, or the af- 
fected muscles may fail to respond altogether to the inten- 
sity of current which can be employed in children. The 
ordinary tests for sensory disturbances cannot easily be ap- 
plied in very young children. There is generally some 
slight diminution in all forms of sensation, but we can only 
state with certainty that the sensations of touch and of pain 
are impaired in all but the mildest cases. In considering 
the imperfect sensation of a very young child we must bear 
in mind that, as Soltman and Westphal have shown, the 
peripheral nerves are not fully developed until several 
weeks after birth. Evidently the entire central and periph- 
eral apparatus conducting sensation is developed later than 
that transmitting motion. 

The appearance of the affected limb changes a little as 
time goes on. In some of the milder forms of obstetrical 
paralysis a complete restoration takes place within a few 
weeks, or a few months, with or without medical interfer- 
ence. In the severer forms the atrophy is apt to increase ; 
the development of the limb is retarded as compared with 
the normal side. It is common enough to have contract- 
ures at the elbows and at the wrists develop in the course 
of a few months, or after a year or more, in those cases in 
which, either from lack of treatment or from the severity of 
the injury at birth, the initial paralysis remains unaltered. 

Pathology. — It is naturally a difficult matter to deter- 
mine the exact changes which take place in any part of the 
brachial plexus in consequence of obstetrical manipulations, 
for the opportunities of a post-mortem examination are 
very rare. Roger describes a case in which the facial 



212 THE NERVOUS DISEASES OF CHILDREN. 

nerve, and the arm of the same side were paralyzed imme- 
diately after birth from pressure of the forceps. Large 
extravasations of blood were found post mortem in the 
vicinity of the stylomastoid foramen, and of the brachial 
plexus. Oppenheim, who examined one of Henoch's cases, 
discovered degenerative changes in the brachial plexus. 
In the milder forms of these peripheral lesions we must 
suppose that the disturbance of function was due to slight 
mechanical injuries, to a stretching or tearing of some of 
the fibres, possibly to slight injury of the nerve-sheath, or 
a mild form of inflammatory reaction in the nerve-tissue. 
In the severer cases the lesion may amount to an actual 
tear, to a severance of the nerve-fibres, or, in the case of 
subluxation of the head of the humerus, to actual compres- 
sion, with loss of function, of the nerve-fibres. The lower 
arm type (Klumpke) of brachial plexus lesion has been re- 
ported. Also a case of laceration of the spinal roots (Phil- 
ippe and Cestan). 

Diagnosis. — The differential diagnosis is to be made 
between these obstetrical palsies and the so-called " birth 
palsies " due to cerebral lesions. A knowledge of the 
symptoms due to lesions of the motor tract will be useful. 

Peripheral Palsies (Obstetri- Cerebral Birth Palsies, 

cal Palsies of Brachial 
Plexus). 

1. Arm only affected. I. Hemiplegia or diplegia common ; 

brachial monoplegia rare. 

2. Flaccid paralysis with atrophy. 2. Spastic paralysis, with or without 

atrophy, with tendency to rigid- 
ity. 

3. Deep reflexes absent, surely not 3. Deep reflexes increased. 

exaggerated. 

4. Changes in electrical reaction from 4. No changes in electrical reaction. 

simple loss of faradic response to 
complete reaction of degenera- 
tion. 

5. No convulsions. 5. Convulsions apt to occur and to 

be repeated. 

6. Deformity and arrested growth of 6. Flexion contraction of fingers, 

entire extremity. wrist, and elbow. 

7. Sensation may be impaired. 7- Sensation not affected. 



DISEASES OF THE PERIPHERAL NERVES. 21 3 

There is no other condition from which these obstetrical 
palsies need to be differentiated if the child is examined 
within a reasonable time after birth ; but if, as is so often 
the case, the child is brought to a physician when it has 
reached the age of six, seven, or even twelve years of age, 
the true cause of the palsy may be difficult to fathom. At 
my clinics I have often seen cases for which nothing had 
been done, and the cause is elicited only after the closest 
inquiry from the mother. In older children it is a question 
whether the palsy dates from birth, or whether some acci- 
dent in the first few years of life may not have been the 
cause. The pulling of the arm in some of the games the 
children play, wrenching and twisting it as they do with 
considerable force, may result in palsies very much like 
obstetrical palsies. Poliomyelitis is not easily to be con- 
founded with these obstetrical palsies, for the former is 
rare in the first year of life, and relatively rare in the upper 
extremities ; the atrophy is generally more extreme, sensa- 
tion is not involved, and complete recovery is not common 
in the spinal paralysis of children. 

Prognosis. — If properly cared for, recovery may be ex- 
pected in all but the most severe forms of obstetrical pe- 
ripheral palsy. According to the severity of the symptoms, 
the length of time that it will take before recovery sets in 
can also be foretold, with some degree of certainty, and 
for this purpose the electrical behavior of the nerves and 
muscles constitutes a fair test for the amount of damage that 
has been done. Even though the paralysis be complete, if 
the faradic response of the branches supplying the affected 
muscles is not entirely lost, the probability is that complete 
recovery will set in within a period of two to three months. 
If the faradic response is lost, but the galvanic formula is 
not altered, the restoration of function may be expected 
within a period of six months ; but if both the faradic and 
the galvanic responses are entirely gone or seriously im- 
paired, it may be a year, or even two years, before the arm 
can be properly used. In the severer cases, in which a 
great deal of injury has been done to the brachial plexus, 
it will not be well to promise much regarding the time or 
the possibility of complete recovery. Some of these cases 



214 THE NERVOUS DISEASES OF CHILDREN. 

» 

are extremely stubborn ; this is to be emphasized, for the 
majority of authors seem to regard these conditions too 
favorably. Much will depend upon the accuracy of treat- 
ment. One point which I wish to impress upon the reader, 
however, is that in a fair proportion of all these cases the 
physician may reassure the parents regarding the future 
condition of the paralyzed extremity. 

Treatment. — If the child is seen immediately after 
birth, and the diagnosis has been properly made, the best 
the physician can do is to let the limb severely alone for a 
period of at least two weeks ; at the end of that time the 
exact damage that has been done can be properly estimated 
and the necessary therapeutic measures can be employed. 
Wrapping up the arm carefully, so as to avoid any further 
injury to it, and allowing it to be bathed as the rest of the 
body is, will be all that is necessary. After a period of 
two weeks I would advise the use of light friction and 
massage, so as to keep up the nutrition of the muscles, and 
I am also in favor of directing an intelligent nurse or rela- 
tive to move the parts systematically so as to overcome 
any tendency to contracture. After a period of four to 
six weeks electricity may be employed, simply as a mode of 
exercise. If the parts respond to a mild faradic current 
such may be employed, but if the faradic current fails to 
elicit a response it is sheer waste of time to employ this 
form of electricity, and it will be better to use a mild gal- 
vanic current, using that pole which will produce contrac- 
tions. Sittings of from five to ten minutes every day, or 
every other day, are quite sufficient, and will be of some 
benefit in the way of preventing excessive atrophy and of 
preventing the formation of contractures. 

As soon as there is a tendency to the development of 
contractures, it will be well to place the arm in a splint 
in such a way as to oppose the contracture by simple me- 
chanical force, and to take the arm out of the splint daily 
for the purpose of treating it by proper massage and elec- 
tricity. If deformities have arisen as the natural result of 
disease, or from neglect, the child should be placed in the 
hands of an orthopedic surgeon and an attempt should be 
made by every known surgical procedure to correct the 



DISEASES OF THE PERIPHERAL NERVES. 21 5 

existing trouble. The old belief that a child will outgrow 
such deformities is absolutely unfounded. It is an unfortu- 
nate fact, but true, nevertheless, that children outgrow only 
the very mildest disturbances of the nervous system, which 
is equivalent to saying that some troubles undergo spon- 
taneous cure. Clark and others have advised suturing of 
the lacerated strands in severe cases ; some favorable re- 
sults have been reported. Great caution should be exer- 
cised in the selection of cases. 

Paralysis of the lower limbs due to obstetrical manipulations is reported 
every now and then, but it is extremely rare. The lumbar and sacral plexuses 
are so well protected at every point of emergence from the spine and in their 
course through the gluteal and pelvic regions that they are not easily disturbed 
by any amount of manipulation or traction upon the legs. An injury of these 
parts is, however, conceivable. If present, the paralysis will resemble the 
paralysis of the adult in the number of muscles involved and in the character 
of the palsy. It is interesting in this connection to note that Ross, many 
years ago, supposed that traction of the legs was occasionally responsible for 
spastic paraplegia in children. He does not refer to the effect upon the 
nerve-roots, but supposes that such traction would be followed by derange- 
ment of the fibres in the spinal cord itself. This view has not been accepted, 
and it has been shown that cases of spastic paraplegia due to traumatism 
during birth are due, in the vast majority of cases, to cerebral and not to 
spinal injuries. Some exceptions have been credibly reported. 

Still another obstetrical palsy occurring quite exceptionally is facial paral- 
ysis due to compression of the peripheral portion of the nerve by the blade of 
the forceps. This is an unusual occurrence, for if the blades of the forceps 
are properly applied they should not be in the vicinity of the facial nerve ; 
but accidents will happen and the blades occasionally slip even if applied by 
skilful obstetricians, and thus cases of obstetrical facial nerve palsy are not 
entirely unknown. This may be the result of actual pressure, and in other 
cases an extravasation of blood into the region of the parotid is likely to com- 
press the nerve sufficiently to cause temporary palsy. Exostoses within the 
pelvis or hasmatomata may cause facial palsies by direct pressure (Vogel, E. 
Kehrer, Schultze). Schiitze and A. Stein have reported facial and hypoglossal 
palsy, due, presumably, to the rare coincidence of an external hematoma and 
a small hemorrhage into the hypoglossal nucleus (during normal labor). 

The prognosis in these cases is, on the whole, favorable. In a few cases, 
however, a degeneration of the nerve occurs in consequence of the severity of 
the compression. Henoch reports two such cases. Parrot and Troisier 
have examined such nerves post mortem. I have the records of two cases, 
one in a girl of twelve, the other in a woman of some twenty-two years 
of age, in whom facial palsy with unequal development of the two halves 
of the face is the result of this obstetrical accident. 



2l6 



THE NERVOUS DISEASES OF CHILDREN. 



The treatment of these cases is exactly the same as that for all other pe- 
ripheral palsies. Light massage and the use of the electric current are the 
only measures that can be safely recommended. 

FACIAL PALSY (BELL'S PARALYSIS). 

Disease of the seventh nerve is not infrequent in children 
from the same causes that result in facial paralysis in the 
adult. We have to con- 
sider, first, rheumatic fa- 
cial palsy, more properly 
perhaps refrigeration pal- 
sy ; second, facial palsy 
due to ear disease ; and 
third, facial palsy as an 
accompaniment of disease 
at the base of the brain. 

Rheumatic facial pal- 
sy may occur in children 
at almost any age. Al- 
though I have seen it in 
a child nine months old, 
it is rare before the age 
of three years, and is most 
frequent between the ages 
of six and fifteen years. 
Sudden draughts of cold 
air are by far the most 

frequent Cause. The Side FlG - 54- -Diagrammatic figure showing the Re- 
f . f -i.i lations of the Seventh and Glosso-Pharyngeal 

of the lace exposed to the 
draught is generally the 
one that is paralyzed. In 
a few instances it is diffi- 
cult to make out the ex- 
act cause, but even under 
such circumstances re- 
frigeration is very prob- 
able. I am not aware that 
facial palsy occurs in connection with the various diatheses 
in children as it does in the adult, in whom it is met with in 
association with diabetes or tuberculosis. 




Nerves and the Course of the Taste Fibres. 
The lines i, 2, 3, 4 mark off different segments 
of the nerve. N. f. , facial nerve ; N. a. , Acous- 
tic nerve ; G. ph. , glossopharyngeal nerve ; 
G. g., geniculate ganglion ; N % p. s., great su- 
perficial petrosal from fifth nerve ; JV. c. c.p. A, 
tympanic plexus ; the dotted line S. p. s. indi- 
cates salivary fibres ; the other dotted line in- 
dicates taste fibres; Ch. /., chorda tympani ; 
N. st, stapedius nerve; F. st., stylo-mastoid 
foramen. (Modified from Erb by Dana.) 



DISEASES OF THE PERIPHERAL NERVES. 2\*J 

The symptoms of facial palsy will be readily under- 
stood by an examination of Fig. 54. The distal portion (1) 
is the one which is generally affected in these rheumatic 
cases. All the branches of the facial nerve are involved, 
but there is no interference with taste, with hearing, with 
movement of the palate, or with the secretion of saliva. 
The paralysis involves the muscles of the eyes, the nose, 
the cheeks, and the lips. On inspection of the child's face it 
is easy to make out which side is affected, as the eyelids of 
the diseased side are wide open (lagophthalmos). If the 
child is asked to close the eyes it does so with ease on the 
sound side, but the diseased side fails to respond.* In 
this effort there is over-action of the frontal muscles and 
of the corrugator supercilii on the sound side ; the diseased 
side showing either diminished action or a total paralysis. 
The child cannot pull up its nose ; in blowing out the cheeks 
the diseased side is flabbier than the sound side, and if the 
attempt is made to whistle or to pout the lips, as in kissing, 
the insufficient action of the muscles on the diseased side 
becomes very evident. If asked to show the teeth, the 
muscles of the sound side overact, and the mouth is drawn 
toward the healthy side. By this movement the paralysis 
is often revealed, even if the parts appear perfectly normal 
while at rest. The tongue when protruded deviates toward 
the sound side ; this deviation was formerly considered to 
be apparent, not real ; but Hitzig has shown that there is 
an actual deviation toward the sound side, and supposes 
that this is due to the fact that the tongue wishes to avoid 
contact with the corner of the mouth on the paralyzed side. 
Contractures of the muscles of the paralyzed side occur in 
the later stages of a facial palsy. The lower branches re- 
cover, as a rule, more slowly than the upper, and in several 
cases I have observed that the lower and middle muscles 
could be contracted only if the attempt was made to close 
the eyes (Fig. 55). 

Sensory disturbances are entirely absent, as the facial 
nerve is a purely motor nerve, the sensory fibres of the face 

* A few cases of double facial palsy have been reported (Mott, Huebschman) ; a suc- 
cessive involvement of the two sides is more frequent. The author has observed it in 
cases associated with diabetes in the adult. 



218 



THE NERVOUS DISEASES OF CHILDREN. 



coming from the fifth nerve. I have seen a few cases of 
severe neuralgic pains complicating facial palsy in children, 
but to account for it there has always been some additional 
cause, as in a boy of fourteen, whom I recently examined, 
in whom decayed upper teeth offered the explanation for 
the neuralgic supraorbital pain. 

Whatever part of the nerve is involved, provided it be 
between the nucleus of the facial nerve in the pons and 
the periphery, electrical changes will be found. In all but 




Fig. 55 — Boy with Facial Palsy, in Stage of Recovery. Slight contracture of para- 
lyzed side (B) ; in figure A mouth is pulled to the paralyzed side when boy attempts 
forcibly to close left eye ; also deepening of naso-labial fold. 



the very mildest cases the faradic response of the nerve 
as well as of the muscles is lost. My own experience has 
proved, however, that there is an exception to this rule, and 
that is, that if the patient be examined within the first forty- 
eight or seventy-two hours after the onset of the palsy, the 
faradic response may still be present ; it rapidly diminishes, 
however, and after a period of three days, in the vast ma- 
jority of cases, it is entirely lost. The galvanic response 
of the nerve may be increased during the first few days, 
but it is soon diminished or lost. The galvanic irritability 
of the muscles supplied by the facial nerve shows many in- 
teresting changes. In the earlier stages of the disease, and 



DISEASES OF THE PERIPHERAL NERVES. 219 

in all but the severest cases, there is an increased galvanic 
excitability for a period of about two weeks. The same 
currents which produce very strong contractions on the 
diseased side give weaker contractions on the sound side. 
This can be easily demonstrated in the case of the orbicu- 
laris oris. If a very weak current be applied to the sound 
half of the muscle, contractions may be seen in the diseased 
half and not in the normal half, the increased excitability 
in the latter being so great that the small amount of current 
diffusing to the paralyzed side is sufficient to produce a 
contraction, while the full current is not sufficient to make 
the healthy half contract. During this period the cathodal 
closure contraction of the diseased muscle is generally 
greater than its anodal closure contraction. After a little 
while the increased excitability diminishes and soon falls 
below that of normal muscular tissue. The polar con- 
tractions also indicate a change, the cathodal closure con- 
traction first being equal to the anodal closure contraction ; 
later on in the severer cases the order is entirely reversed. 

As the disease approaches the period of recovery the 
galvanic formula approaches more nearly to the normal, 
the cathodal closure contractions being equal to the anodal 
closure contractions. Finally, the cathodal closure con- 
traction becomes greater than the anodal contraction, and 
the contractions that have been sluggish once more become 
prompt and short. The faradic response is, as a rule, rather 
longer than this in returning, and it is not uncommon to 
see the full power of all the muscles return before the nor- 
mal electrical reactions have been established. In the case 
of a boy (Fig. 55) the faradic response of the muscles and 
a normal galvanic formula were not restored until several 
months after the contractures had set in, and after most of 
the muscles could be innervated tolerably well. 

In facial palsy due to ear disease, the symptoms can 
be easily accounted for by the position of the nerve in the 
Fallopian canal. The nerve may be involved during 
an attack of otitis media, and as this ear affection occurs 
often enough in children in association with several 
acute infectious diseases, notably scarlatina, this form of 
facial palsy is quite a frequent occurrence. It does not 



220 THE NERVOUS DISEASES OF CHILDREN. 

occur as readily in the earlier stages of an otitis as in the 
later suppurative stages, when, in consequence of caries of 
the bone, the facial nerve is directly involved. According 
to the severity of the disease the paralysis will be more or 
less complete, and as far as the facial nerve is concerned 
the symptoms will resemble those of the rheumatic form, 
but naturally the association of facial palsy with defective 
hearing or the persistence of a purulent discharge from the 
ear will point to the actual cause of the paralysis. 

I have seen facial palsy occur also after mastoid opera- 
tions for ear disease. In these cases an accident during the 
operation is often sufficient to break through the fine bony 
plate separating the facial nerve from the ear structures, 
and a more or less severe facial palsy is the result. This 
occurred in a little patient whose case I reported fifteen 
years ago, in whom the mastoid operation was done for the 
relief of epileptic attacks which were caused by the reten- 
tion of pus. On his recovery from the operation a distinct 
facial palsy on the side of the operation was noticed, and ex, 
isted for a period of very nearly three months, after which 
complete recovery took place. 

As in the adult so in the child, paralysis of the face may 
accompany lesions at the base of the brain and in the pons. 
If the lesion is at the base, other cranial nerves are involved 
as well as the facial, and the diagnosis can therefore be 
readily made. It is common enough to find the facial 
nerve partially or totally paralyzed in cases of basilar men- 
ingitis, whether tubercular or non-tubercular. In cases of 
injuries to the skull, or of tumors occupying the middle or 
posterior fossa, facial palsy is one of a large series of symp- 
toms and has but little diagnostic value excepting in its as- 
sociation with affections of other cranial nerves. It may be 
stated in this connection that in cases of cerebellar tumor, 
which are by no means rare in children, both the acoustic 
and the facial nerves, which lie in very close juxtaposition 
to each other and are easily pressed upon by a cerebellar 
growth, are often paralyzed and give rise to symptoms 
which are almost pathognomonic of cerebellar disease. If 
the lesion is in the pons, and particularly if it be below 
the decussation of the facial nerves, paralysis of the face is 



DISEASES OE THE PERIPHERAL NERVES. 221 

associated with alternate hemiplegia. In these cases every 
branch of the facial nerve is apt to be involved. If the 
lesion be a neoplasm other symptoms pointing to compres- 
sion of the cranial nerves and indicating the presence of 
tumor within the cranial cavity will naturally be associated 
with those mentioned before. 

The diagnosis of facial palsy is easy to make, and the only difficulty that 
arises at tiroes is to determine whether the palsy is indicative of a central or 
a peripheral lesion ; but this difficulty can be readily met by keeping in mind 
the fact that if the lesion is in the brain anywhere above the pons the upper 
branches of the facial escape altogether and the electrical responses remain 
entirely normal ; furthermore in such a case the facial palsy is associated with 
hemiplegia on the same side of the body as is the paralysis of the face. It is 
far more difficult to determine the exact part of the nerve that is diseased. 
As the intensity of the affection depends somewhat upon the extent of the 
nerve tract that is diseased, this inquiry into the accurate localization of the 
disease has a practical as well as a scientific interest. Taking the diagram 
on page 216 we can study the variation of symptoms according to the part 
that is affected. If the disease is limited to that part outside of the Fallo- 
pian canal, there is complete paralysis of every branch of the facial nerve 
with the exception of the posterior auricular. Reflexes are wanting, but there 
are no disturbances of taste or hearing, no paralysis of the palate, and no dis- 
turbances of salivary secretion. If the disease happens to affect the part of 
the facial nerve as far as, but not including, the chorda tympani (Fig. 54, line 
2), the symptoms are paralysis of all the branches of the facial, including 
the posterior auricular. 

The same symptoms as the above, with the addition of a disturbance of 
taste in the anterior portion of the tongue and a diminution in salivary secre- 
tion points to an involvement of the facial nerve from the point of junction 
of the chorda tympani to the junction of the stapedius with the main trunk 
(from 2 to 3). 

If that part of the nerve lying between the stapedius and the ganglion 
geniculi is diseased, the symptoms, in addition to total paralysis, are changes 
in taste, diminution in salivary secretion, and abnormal acuity of hearing, but 
there is no paralysis of the soft palate. The above symptoms, with the addi- 
tion of paralysis of the soft palate, point to a lesion in the region of the gen- 
iculate ganglion (division 4 to 5) ; but on this point there is much doubt. 
See table, p. 13. 

Paralysis of all the peripheral branches, as well as paralysis of the soft 
palate, abnormal acuity of hearing, decreased salivary secretion, but no involve- 
ment of taste, help to locate the lesion above the geniculate ganglion, and be- 
tween it and the exit of the facial nerve from the brain (division 5 to 6). If this 
part of the facial tract is diseased other cranial nerves are frequently affected. 
In such cases we are apt to have paralysis of the abducens, loss of hearing on 
the same side as the facial palsy, tinnitus aurium, and the reaction of degen- 



222 THE NERVOUS DISEASES OF CHILDREN. 

eration of the acoustic nerve. If we have total palsy of every branch, palsy 
of the soft palate without disturbance of taste and with simple diminution of 
electrical excitability, without any other marked symptoms, we may locate 
the lesion in the facial nucleus ; but this form of palsy is recognized more 
definitely if other cranial nerves are simultaneously affected, such as the 
hypoglossal, the spinal accessory, the vagus, the trigeminal nerve, etc. 

The association of the symptoms just mentioned with paralysis of the arm 
and leg of the opposite side, puts the lesion in the pons. If there is but par- 
tial paralysis of the face, if the electrical excitability is not affected, if the re- 
flexes are normal, and if the extremities of the same side are affected, the 
lesion is in the crus or in the hemispheres, say in the internal capsule. If the 
facial nerve and the oculo-motor nerve of opposite sides happen to be affected 
the lesion is positively in the crus on the same side as the ocular palsy. 

The course and prognosis of all forms of facial palsies 
will depend entirely upon the nature of the morbid proc- 
ess. If the disease is due to caries of the petrous por- 
tion of the temporal bone, to malignant tumor, to fracture, 
to tubercular meningitis, or to any form of meningitis, the 
prognosis is extremely grave. Even those cases depending 
upon specific disease at the base are not as amenable to 
treatment as one might expect. Those due to parotitis or 
to internal otitis recover if the disease itself is not of the 
destructive type. Cases due to simple section of the 
nerve, as in cases of operation, yield a tolerably favorable 
result. 

The prognosis of the rheumatic forms of facial palsy 
varies according to the intensity of the disease, but it does 
not depend apparently upon the amount of nerve-tissue 
that is affected, for, on the whole, cases in which the nerve 
within the canal is involved take about as favorable a 
course as do those in which the pes anserinus is the only 
part affected. Erb distinguishes three forms — a mild form, 
a moderate form, and a severe form of facial paralysis. His 
division depends entirely upon the electrical conditions 
present at the end of the first week. I cannot agree alto- 
gether with his statement that if the electrical excitability 
is entirely normal at the end of the first week, as in light 
forms, recovery will set in in two or three weeks. If at 
this same period the faradic and galvanic excitability are 
slightly diminished recovery may be expected in from four 
to six weeks, and if at the end of the first week the elec- 



DISEASES OF THE PERIPHERAL NERVES. 223 

trical excitability of the nerve is very much diminished or 
totally lost, the disease will run a course of many months. 
I have seen cases get well practically within four weeks, in 
which the faradic and galvanic excitability of the nerve 
was diminished very much from the very start, and I 
should be inclined to formulate the following statements : 

I. If at the end of the first week, or, still better, at the 
end of the second week, the nerve responds at all to the 
faradic or galvanic current, a prompt recovery in about 
four weeks may be expected. 

II. If at about the same time the nerve fails to respond, 
but the muscles show a diminished or altered galvanic re- 
sponse, the disease is likely to run a course anywhere be- 
tween one and three months. 

III. If the muscles respond but feebly to strong cur- 
rents, if the galvanic formula is altered, and if the contrac- 
tions are extremely slow, the disease may run a course any- 
where between six months and a year, or even longer. If 
after a period of two months no electrical reaction can be 
observed, the degeneration is very complete, and a paral- 
ysis lasting at least a year, if not longer, may safely be pre- 
dicted. 

Treatment. — We need say little of the treatment in cases 
in which the facial palsy is only one of many symptoms 
pointing to gross cerebral disease. Under such circum- 
stances treatment directed to the relief, or possibly the cure, 
of these conditions will have to be employed. In the cases 
of facial palsy due to ear disease, proper surgical treatment 
should be recommended at a very early day. Much doubt 
has been expressed as to whether any treatment can cur- 
tail or cure the rheumatic palsies. The milder forms will 
undoubtedly get well even if no treatment is attempted. 
In the more chronic cases the use of the electrical current, 
particularly of the galvanic, can be safely recommended, 
for I have the distinct impression that cases so treated, and 
particularly those that have been properly treated from the 
very start, run a somewhat shorter course than those in 
which no electrical treatment has been attempted. 

In the year 1893 Remak gave statistical evidence in favor 
of this view, and put a quietus for the present upon those 



224 THE NERVOUS DISEASES OF CHILDREN'. 

who think that there is nothing in electro-therapeutics save 
the effect of suggestion. 

The electrical current, even if it have but slight influ- 
ence in restoring the power of conduction in the diseased 
nerve, is surely of value, as it is in paralysis of the extremities, 
in exercising parts that cannot be moved by the will, and for 
this reason if for no other it deserves to be employed. 

Counter-irritation has been repeatedly tried. This may 
be accomplished by blistering either with emplastrum can- 
tharidum just in front of the ear or over the mastoid, or by 
the use of the actual cautery. I am free to confess, however, 
that the value of counter-irritation seems questionable in all 
cases, and particularly in those in which counter-irritation is 
not indicated to allay pain that is so frequently associated 
with neuritis. The application of leeches is to be thoroughly 
condemned, except possibly in those cases in which the fa- 
cial palsy is associated with ear disease. This practically 
limits us to the use of electricity as the only therapeutic 
measure which promises some relief in the severer cases. 
In these cases the faradic current, as recommended by 
Erb and Duchenne, may be employed for the first week 
or two, but after that a stabile galvanic current applied 
over the mastoid and along the peripheral divisions of 
the facial nerve is more in order. As soon as the ex- 
citability of the nerves and muscle is increased, or shows 
an approach to the normal, regular electrizations of the 
paralyzed muscles, in sittings of ten minutes or more, two 
or three times a week, should be practised. I wish to cau- 
tion the physician, however, against the use of strong cur- 
rents or against the use of the electrical current without 
the safeguard of a galvanometer. A current varying be- 
tween two and four milliamperes is quite sufficient for all 
purposes. I have also directed my patients, as soon as the 
least power returns, to practise contraction of the muscles 
(closing of the eyes, pouting of the lips, etc.), while look- 
ing into a mirror. 

As for medicinal measures, they may be discarded alto- 
gether. In many cases I have tried strychnia both per os 
and in the form of hypodermic injections, and I am firmly 
convinced that the effect of such medication is absolutely 



DISEASES OF THE PERIPHERAL NERVES, 225 

nil, nor can we expect much more from the use of irritat- 
ing ointments or douches to the face. If drugs must be 
administered one may exhibit iron, arsenic, and cod-liver 
oil. In inveterate cases, union of the facial with the spinal 
accessory or the hypoglossal nerve may be attempted. 

Other Peripheral Palsies. — Almost any peripheral palsy which is 
known to occur in the adult may at times occur in the child. Of these I will 
make special mention only of a peripheral palsy, generally of traumatic ori- 
gin, affecting the peroneal nerve. Injury to this nerve occurs readily enough 
if a child happens to be violently struck upon the leg, or if the leg happens 
to be squeezed against a post, a chair, or, as in one case I have seen, against 
the edge of the bed. I mention this special palsy because it may lead to 
slight difficulties in diagnosis, inasmuch as confusion may arise with acute 
anterior poliomyelitis which affects the muscles supplied by the peroneal 
more often than those supplied by any other nerve. 

The onset of the paralysis without fever, the presence of considerable 
pain, the marked and rapid tendency to recovery, and the history of violent 
traumatism will help to distinguish this peripheral palsy from the essential 
paralysis of children. The course, prognosis, and treatment of such a pero- 
neal neuritis will be exactly the same as in the case of other nerve palsies. 

Spasm of various peripheral nerves is common in young persons. The 
facial and the spinal accessory nerves are frequently affected ; the trigeminal 
in rare instances only. Spasm of the facial nerve (tic convulsif) is much 
less frequent than in adults ; a spasm involving an entire facial nerve of a 
child is very rare indeed ; but partial spasms are observed in the form of nic- 
titation (clonic spasm) or of blepharospasm (tonic spasm of the obicularis 
palpebrarum). These movements are generally due to the influence of cold, 
to irritation of the trigeminus, but are also frequently of psychic origin. 
In the cases of clonic spasm of the entire nerve, all sorts of extravagant 
grimaces are indulged in. These spasms must not be confounded with habit 
chorea. 

Spasm in the distribution of the spinal accessory nerve involves either the 
sternocleido-mastoid or the trapezius (spasmodic wryneck). 

The spasm may be reflex in origin, or due to irritation of the nerve in its 
course from the medulla and spinal cord to the periphery. The position and 
movements of the head will vary according to the muscles involved. These 
spasms may be unilateral or bilateral. 

If we remember that spasm, whether clonic or tonic, denotes an excess of 
normal function, the symptoms can be easily understood by reference to the 
tables on pages 15 and 16. 

Ordinary wryneck, so frequent in children, is due to a rheumatic myo- 
sitis. Symptomatic wryneck occurs in connection with cervical adenitis, 
abscesses and caries of the cervical spine. Congenital wryneck is due to 
some pre-natal disturbance ; it may be of intra-uterine origin, and denote im- 
perfect development, in which case it is generally associated with atrophy 



226 THE NERVOUS DISEASES OF CHILDREN 

of one-half of the face ; at times it is due to obstetrical injuries in cases of 
breech and foot presentations. 

The treatment of spasms of the peripheral nerves consists in the use of sed- 
atives, such as opium, conium, codein, chloral, and bromides ; in some cases 
the valerianate of zinc, or the ammoniated tincture of valerian, might be given. 
The galvanic current may be applied in the milder cases. The attempt may 
be made to check the spasms by hypodermic injections of hydrobromate of 
hyoscine, one two-hundredth to one one-hundredth grain, or by the use of 
atropia (one-fiftieth to one-tenth grain, as recommended by Leszynsky). 

Tenotomies are in order in the congenital and chronic cases. Keen has 
suggested resection of the posterior branches of the upper three or four 
cervical roots ; but the advisability of this procedure is still in doubt. 

Spasm of the hypoglossal nerve I have not seen in children. The com- 
plex co-ordinated spasms have been considered in Chapter VI. 



CHAPTER XIII. 

MULTIPLE NEURITIS. 

Neurology has few more signal achievements to show 
than the advances that have been made in the study of 
multiple neuritis within the past decade. Many conditions 
which were formerly supposed to be due to an affection 
of the spinal cord, and which were often confounded with 
poliomyelitis, and with various forms of acute and sub- 
acute myelitis, are now positively known to be due to poly- 
neuritis. The term multiple neuritis or polyneuritis is 
used to embrace all those diseases in which the symptoms 
are due to disease of several or many of the nerves of the 
body. There is not only a tendency to multiplicity but 
also a tendency to symmetrical development. 

Many of the older writers recognized the possibility of multiple nerve af- 
fections, and a multiple neuritis, as such, was recognized by Dusmenil as far 
back as 1864, and even a few years earlier Virchow had described a multiple 
neuritis occurring together with leprosy. Going back still further, the histor- 
ical investigations have led to the discovery that Dr. Jackson, of Boston, had 
recognized peripheral multiple neuritis as early as 1822; but all these re- 
searches, even including those of Duchenne, did not help to place the knowl- 
edge of multiple neuritis upon a firm basis. The subject was revived by Jof- 
froy, in 1879, an d Leyden, in 1880, and since that time innumerable articles 
have appeared by writers of almost every nationality. 

Comparatively few writers, however, have had much to say regarding 
multiple neuritis occurring in children. Diphtheritic paralysis, to be sure, was 
discussed clinically long before it was known that it was due to peripheral 
neuritis. Multiple neuritis is not so frequent in children as in adults, for the 
simple reason that the causes leading to it are not as powerful in early life as 
later on ; and yet cases are common enough to necessitate a discussion of 
them in this book. 

Symptoms. — Whatever the cause of the multiple neuritis 
may be the symptoms are, with few modifications, practi- 



228 



THE NERVOUS DISEASES OE CHILDREN 



cally the same in all cases. Though the development of the 
various symptoms is subject to great variations, the symp- 
toms are such as we might well expect on physiological 
grounds from disease of the peripheral mixed nerves. The 





Fig. 56.— Young Boy with Multiple Neuritis, showing Double 
Slight " Foot Drop." 



Wrist Drop " and 



most characteristic feature of multiple neuritis is the asso- 
ciation of motor paralysis with sensory paralysis, the dis- 
tribution of each harmonizing with the other and showing 
very definite anatomical limits. The paralysis is of the 
flaccid order, leads at an early date to atrophy of the af- 



MULTIPLE NEURITIS. 2 29 

fected muscles, and the electrical conditions are altered so 
that we may find almost every possible form of the reac- 
tion of degeneration from a mere loss of faradic irritabil- 
ity to an absolute loss of galvanic response on the part of 
the nerves and muscles. The distribution of the paralysis 
is, as a rule, entirely symmetrical, and may affect either 
the upper or lower, or all four extremities ; it may involve 
every part of all the extremities, and is the one affection 
which, perhaps more frequently than any other, leads to 
a complete paralysis of every limb of the body. It is one 
of the diseases, therefore, which the physician should bear 
in mind if he finds a patient who is totally paralyzed with 
the bare exception of the head and tongue, the jaws and 
eyes ; sometimes some of the latter parts may be paralyzed 
as well. Certain groups of muscles are more readily af- 
fected than others ; thus we find that the extensors of the 
wrist and the extensors of the feet are very frequently in- 
volved, and at an early period of the disease " wrist-drop " 
and " foot-drop " are characteristic phenomena of the dis- 
ease. With the paralysis sensory symptoms go hand in 
hand ; paralysis associated with pain may be noted in some 
cases of spinal-cord disease, as in the acute stages of a 
poliomyelitis, but the persistence of pain in those parts 
which are paralyzed, and along the nerve-tracts, points very 
strongly to a multiple neuritis. Whenever we suspect that 
a paralysis or a deformity may be the result of a multiple 
neuritis, the previous occurrence of a painful affection and 
the duration of the same must be determined with "the 
greatest care. If the patient is seen during the acute stage of 
the disease the tracts of the peripheral nerves will be found 
painful, and this objective proof will be added to the sub. 
jective symptoms which the patient describes. But the ab- 
sence of pain, as evidenced by cases reported by Barrs, 
need not militate against the diagnosis. 

The motor and- sensory paralysis is not developed as rapidly as is the 
case in central affections. There is a premonitory period during which 
numbness, tingling, sensations of heat or cold, in the parts to be affected 
later on, are the annoying symptoms, and after a premonitory period that 
may vary from a few days to a few months the development of the paralysis 
may be acute, subacute, or chronic. In the majority of the cases it is sub- 



23O THE NERVOUS DISEASES OF CHILDREN. 

acute or chronic, and it often requires a period varying between one and two 
weeks until the weakness of the parts has advanced far enough to be con- 
sidered an actual paralysis. The onset is marked occasionally by the oc- 
currence of fever, and if the disease is due to any toxic agent that has con- 
stitutional effects the beginning may be signalized by convulsions as well as 
by rigors. Under the latter circumstances the symptoms may very closely 
resemble those of an acute poliomyelitis, but it is rare, after all, to find as 
stormy an onset in cases of multiple neuritis as in spinal infantile paralysis. 

Before the paralysis becomes absolute, if it ever reaches 
that stage, tremor of the parts and inco-ordination may be 
associated with loss of power and loss of sensation. The 
patient may be able to stand or walk, but does so imper- 
fectly, sways with the body when attempting to stand, and 
may fall if the eyes are closed. The reflexes connected 
with the parts affected by the paralysis are almost inva- 
riably diminished or lost ; this is particularly true of the 
knee-jerk, since the trouble so often begins in and involves 
the legs to a greater extent than any other part of the 
body. The reflex is often absent before the paralysis is 
developed. An exception to the rule of loss of knee-jerk 
has been reported by Strumpell. A few years ago he de- 
scribed a case in which the reflexes were increased, and 
this increase he attributed to irritation of the afferent por- 
tion of the reflex arc. 

The more distal parts of the extremities are, as a rule, 
the first to be affected. In the lower extremities the ear- 
lier stages of the disease are marked by an inability to 
raise the toes while the heel remains on the ground, show- 
ing a weakness of the extensors of the toes, and implying 
a loss of function of the branches of the anterior tibial 
nerve. It is a curious fact that the muscles supplied by 
the anterior tibial nerves are not only most frequently af- 
fected in multiple neuritis, but they also innervate the re- 
gion which is frequently involved in poliomyelitis. In the 
upper extremities the extensors in the forearm are affected 
in the earliest stages of the disease, and from these parts 
the paralysis may extend upward in both extremities until 
the greater number of the muscles are affected or until total 
paralysis of all the extremities is established. The thenar 
and hypothenar groups are not affected at the outset. It 



MULTIPLE NEURITIS. 23 1 

will be seen from this that the musculospiral nerve in the 
upper extremity, and its homologue, the peroneal nerve, in 
the lower extremity, are the nerves first attacked by the 
disease in a large number of the cases. This peculiar pre- 
disposition of these nerves to the disease cannot be ex- 
plained on the ground of anatomical position, and for the 
present is as inexplicable as are the facts of elective affin- 
ities in so many other diseases. 

In some cases the nerves supplying the trunk muscles, 
including the diaphragm, may be affected, but this is, after 
all, a very great rarity. A paralysis of the vocal cords 
due to multiple neuritis is rare enough, as is also an involve- 
ment of the facial nerve and of the hypoglossal. I have, 
however, seen a number of cases, particularly after diph- 
theria, in which the ocular muscles, the vocal cords, and the 
muscles of deglutition were paralyzed. 

The paralysis, although it may involve a number of groups of muscles, 
does not affect all equally ; those less affected may undergo contracture, such 
contractures occurring quite as often in the lower limbs as in the upper 
extremities. Deformities quite as serious and quite as persistent as those in 
poliomyelitis may therefore result from a chronic multiple neuritis. 

As regards the sensory symptoms it is important to 
note that every form of sensation is about equally impaired ; 
touch, pain, temperature, and muscular sense may suffer 
equally except in the earlier stages, in which a hyper- 
esthesia is a more common than hypaesthesia. In a few 
instances the pain sensibility may be diminished while the 
sensation of touch remains, and even if the sensibility to 
pain is intact the conduction of pain impressions may be 
very much delayed. While pain and paralysis are the dis- 
tinguishing symptoms of a multiple neuritis it should be 
remembered that in not a few instances one or the other 
may be absent, and in some cases pain may never have been 
present to any marked degree, or may have lasted for so 
short a time as compared with the paralysis that the pa- 
tient who is examined at a later stage of the disease fails to 
remember that pain was ever present. The electrical re- 
actions may exhibit every form of change from a very 
slight diminution of faradic and galvanic response to a 



232 THE NERVOUS DISEASES OF CHILDREN. 

partial or complete reaction of degeneration. Atrophy is 
also a prominent and early symptom. The entire absence 
of bladder and rectum symptoms is of great importance in 
the diagnosis of multiple neuritis. Exceptions to this rule 
have been recorded by Oppenheim. 

Vasomotor changes in the paralyzed parts constitute a 
frequent symptom of multiple neuritis. GEdema and glos- 
siness of the skin, which are observed in the majority of the 
cases of peripheral nerve disease, are present in many cases 
of multiple neuritis. If the hand is involved there is a pe- 
culiar tapering of the fingers, which is quite characteristic 
of disease of the peripheral nerves. 

The course of the disease will vary very much according 
to the intensity of the cause. As a rule the symptoms in- 
crease during the first four to six weeks and then diminish. 
While the disease is increasing sensory symptoms are par- 
ticularly prominent. As soon as the acme of the disease 
has been reached these symptoms become less troublesome. 
The motor paralysis is recovered from much less quickly 
than are the disturbances of sensation. This is due not 
only to the actual loss of innervating power but to the 
atrophy of the muscles and to the contractures which have 
been formed. But even in severe cases a favorable change 
sets in sooner or later. The atrophy diminishes and the 
strength of the paralyzed limbs slowly increases until com- 
plete recovery is established. 

In some instances the onset of the disease is a very sudden one. The 
paralysis spreads rapidly and may resemble the course of Landry's paralysis ; 
but the fact that in multiple neuritis the trunk muscles generally escape and 
that the paralysis jumps from the lower extremities to the upper without in- 
volving the trunk, will help to differentiate it from an acute ascending palsy. 
While we have good reason to expect every case of multiple neuritis in a 
child to lead to recovery, death may result in consequence of complicating 
conditions, such as paralysis of respiratory muscles, or of the heart from 
disease of the vagus. Bronchitis and pneumonia are complications very 
much to be feared in children who have any form of multiple neuritis. Cir- 
rhosis of the liver and gross cerebral disturbances, due to alcoholism, which 
are common causes of death in cases of multiple neuritis in the adult, do not 
play a similar rdle in children. On the other hand, the involvement of the 
kidneys in cases of diphtheritic palsy is a frequent cause of death. The 
psychoses described in connection with the multiple neuritis of adults are not 
met with in children. 



MULTIPLE NEURITIS. 233 

Multiple neuritis may result from a number of different 
causes: 1. Toxic forms, including those due to metallic 
and non-metallic poisons. Among the former lead, arsenic, 
and alcohol are the most potent; and in rare instances 
mercury, carbonic oxide, and phosphorus may bring on a 
neuritis. In a very careful article in Keating's " Encyclo- 
paedia," Dr. Putnam, of Boston, has discussed the palsies of 
children due to lead and arsenic. He finds that poisoning 
by arsenic occurs most frequently through the mixtures 
used to destroy vermin, such as " Rough on Rats," " Paris 
Green," " German Fly-paper," and the like. Furthermore, 
arsenic is contained in many of the coverings used for 
books, toys, confectionery, labels, etc., as well as for arti- 
ficial flowers. The same author also believes that wall-paper 
and some special forms of carpet may be the source of poi- 
soning by arsenic. The arsenic is supposed to be commu- 
nicated to the child in the form of dust distributed in the 
currents of air, or in the form of some volatile compound 
which may be formed in a room which contains arsenious 
wall-paper. 

That some cases occur from the continued administration of arsenic in 
diseases like chorea there is very little doubt, and it is well to watch children 
carefully, while they are undergoing this form of treatment * As for lead, it 
may enter the system from glazed cards, acid fruits and vegetables put up in 
tins, also from chocolate and other articles wrapped in tinfoil. Lead also 
enters largely into the composition of rubber, and has been found in the rub- 
ber nipples of nursing-bottles. Various kinds of cakes and candies are 
treated with lead, and prove a prolific source of poisoning in children ; but 
children as well as adults are exposed to lead-poisoning very much more fre- 
quently through drinking-water than in any other way. This is more com- 
mon in country districts, where lead pipes are connected with pumps, or 
where lead pipes are used to convey water from wells or springs at varying 
distances to the house. In the large cities this special source of lead-poison- 
ing is extremely rare. 

Alcoholic poisoning in children would naturally be con- 
sidered a rare condition, and so it fortunately is : but 
among the ignorant classes of every large population par- 
ents will be found who take special delight in the fact that 
their child can partake of everything, including beer or 

* I have seen one case of neuritis in the adult after the use of an arsenic paste over 
a large denuded surface. 



234 THE NERVOUS DISEASES OF CHILDREN. 

whiskey. A case of alcoholic neuritis in a child five years 
old was reported years ago from StriimpeH's clinic. The 
child was raised in the saloon of its father, and not only 
drank considerable wine, but also two quarts of beer, 
every day. The father is quoted as saying that " the child 
was always thirsty, and of course we could not give it 
water/' 

2. The toxaemic forms of multiple neuritis are far more 
important — at least in children. Among these we class, first 
of all, the septic forms of multiple neuritis which may occur 
in children as in adults after injury of any sort. The inva- 
sion of micro-organisms is unquestionably the direct cause 
of the neuritis. 

A similar invasion of specific organisms would account for the neuritis of 
leprosy and of beriberi, but these are so rare in this country, and particularly 
rare in children, that we need not make any further mention of them. 

Malarial neuritis is of great interest to us, for though it 
has been described chiefly by authors in Eastern countries, 
it is not unknown in these parts. I have seen several cases 
of this description in children who were brought to my 
clinic from the swampy parts of Long Island. 

Dr. Browning, of Brooklyn, has reported several such 
cases, which were supposed to be cases of poliomyelitis. 
These cases resemble poliomyelitis in their onset and devel- 
opment, and may be easily mistaken for it. In its general 
symptoms this form differs but very little from other cases 
of multiple neuritis except that it is associated much more 
frequently with the intermittent form of fever, that the dis- 
ease itself undergoes marked remissions, and that the en- 
largement of the spleen and examination of the blood are 
likely to give positive proof of the origin of the neuritis. 
Recovery is a little more rapid, too, in these cases than in 
those due to other causes. 

Among the toxaemic conditions which give rise to mul- 
tiple neuritis we must include those blood states which fol- 
low upon the various acute infectious diseases (diphtheria, 
small-pox, typhoid fever, tuberculosis, and also syphilis). As 
the nerve affection is developed a long time after the initial 
infection it is probable, in the light of recent bacteriological 



MULTIPLE NEURITIS. 235 

researches, that some chemical product of the micro-organ- 
isms, and not the bacteria themselves, are the direct cause 
of the neuritis. With the exception of diptheritic multiple 
neuritis (p. 240) all the forms just mentioned are compara- 
tively rare in children. 

In obedience to the ordinary belief, we must concede the 
existence of the rheumatic forms of multiple neuritis; but I 
am free to confess that I have seen only a very few cases of 
multiple neuritis in children which could with some degree 
of probability be referred to rheumatic causes. In cases of 
articular rheumatism in children the nerves are sometimes 
involved, as is proved by pains radiating down the limb 
through special nerve-trunks. Refrigeration neuritis oc- 
curs in children. In one instance this was due to ice-cold 
baths which a mother gave to her child daily all the year 
around. Now and then cases come under one's notice of 
severe forms of polyneuritis in which the true cause cannot 
be ascertained. Specific and tuberculous forms of multiple 
neuritis are rare indeed. 

Pathological Anatomy.— It is natural to infer that 
the changes are very much the same as those to be found 
in cases of ordinary simple neuritis (see page 209). In mul- 
tiple neuritis the changes are chiefly of a parenchymatous 
order, or interstitial in character; the perineurium is not 
often involved. In some cases, however, the nerve is swol- 
len, the nerve-sheath is hyperaemic, of a deep purplish 
color, and, according to Eichhorst, may be covered with 
minute hemorrhages. There may be some slight changes 
also in the consistence of the nerve. All these conditions, 
and the appearances of degeneration, will vary according 
to the stage of the disease at the time of examination. In 
the earlier stages Ave often find the nuclei of the fibres en- 
larged, a proliferation of the connective tissue between 
the nerve-elements, and numerous round or spindle-shaped 
cells with myeline granules near the sheath of the nerve. 
If the specimen is examined at a later period of the disease 
the acute condition will have passed and the nerve presents 
the simple appearance of subacute or chronic degeneration 
of nerve-fibres. (Fig. 57.) 

In some cases the muscular tissue is slisrhtly involved, 



236 



THE NERVOUS DISEASES OF CHILDREN. 



the fibres are smaller and paler, the transverse striation is 
less distinct than under normal conditions, while the nuclei 
of the fibres and of the interstitial tissues may be increased. 
The changes in the muscle may be both parenchymatous 
and interstitial. 

In considering- the pathology of multiple neuritis, the 
most striking fact is the peculiar action of toxic agents upon 
the peripheral nerves, without affecting the spinal centres. 
The farther away the nerve-fibre is from the mother-cell 
the less able it is to resist morbid agencies ; for this reason 
the more distal parts of the peripheral nerves are affected 
at an earlier period in multiple neuritis than are the parts 
of the nerve nearer 
the spinal centres. 



This argument is 
strengthened by the re- 
cent conceptions of the 
close relationship between 
the ganglion-cell and the 
peripheral nerve -fibre as 
parts of a neuron. More- 
over, recent researches 
have shown that the same 
poison which often affects 
the peripheral nerve-fibre 
may occasionally involve 
the ganglion-cell. Stieglitz 
has proved this to be the 
case in lead-poisoning. He 
found a distinct poliomy- 
elitis in animals compelled 
to inhale lead salts. Oppenheim has described slight changes in the spinal 
cord in cases of alcoholic neuritis. Recent investigators have found changes 
in the spinal and cranial nerve roots, and even in the nerve elements of the 
brain (Marinesco, Schlesinger). It is certain that in man lead may cause 
disease of the peripheral nerves. 

DIAGNOSIS. — With our present knowledge of the charac- 
teristic symptoms of multiple neuritis there should be no 
difficulty in differentiating between the various forms of 
this disease and other diseases with somewhat similar 
symptoms. The chief characteristic of these peripheral 
nerve diseases is the association of sensory with motor 




Fig. 57. — Chronic Interstitial Neuritis with Atrophy of 
some Nerve-fibres. (Ziegler. ) a, cross-sections of 
normal nerve-fibres ; b, cross-section of thin, but nor- 
mal, nerve-fibres ; c, endoneurium ; d, cellular in- 
filtration and proliferation of connective-tissue strands 
and blood-vessels ; f, thickened endoneurium with 
small empty nerve-spaces and some normal thin nerve- 
fibres ; g, longitudinal section of a blood-vessel. 



MULTIPLE NEURITIS. 237 

symptoms, the distribution of both pointing to an in- 
volvement of the same nerve-areas. Persistent sensitive- 
ness of the nerve-tracts and subjective pain in the course of 
the peripheral nerves help to corroborate the diagnosis. It 
is easy to mistake multiple neuritis for poliomyelitis, and 
until recent years this mistake was made by the ablest diag- 
nosticians ; but in poliomyelitis the initial symptoms are 
of a rather more violent character, and the entire central 
nervous system often shows the influences of the disease 
very much more than it does in multiple neuritis ; the 
paralysis is more symmetrical in neuritis than in poliomye- 
litis. 

In poliomyelitis there is no pain along the nerve-tracts and these nerve- 
tracts are not sensitive, but vague pains are very often present in the first 
week or two of poliomyelitis. [ have the record of a child, two years old, 
whom I saw two days after the onset of the paralysis, in whom the entire 
limb was so painful that the child would scream violently if the limb was 
suddenly touched, and this was not due to the fear of examination. The pain 
was not distributed along any nerve-paths, and I did not hesitate to make 
the diagnosis of poliomyelitis, which proved to be the correct one by the 
subsequent course of the disease. The electrical reactions are very much 
the same in both disorders. 

The difficulties of this problem are increased by the fact that, as Gowers 
has reported, cases occur in which a typical anterior poliomyelitis is compli- 
cated by a peripheral neuritis. In spite of all efforts at differentiation cases 
will occur every now and then in which it will be impossible to make an ac- 
curate diagnosis at the start, and the physician must watch the course of the 
disease before coming to a definite conclusion. 

From pachymeningitis, which is rare in children, the disease may be dif- 
ferentiated more easily, since in the former the legs are rarely affected, the 
anaesthesia is apt to spread to the upper limbs and to the trunk of the body, 
and there is no distinct sensitiveness to pressure on the part of the larger 
nerve-trunks, except late in the disease, and the atrophy is restricted to 
muscles innervated from the diseased spinal segment. 

Landry's paralysis is to be recognized by the rapidly ascending palsy pro- 
ceeding from segment to segment, involving the abdominal and thoracic 
muscles, and not skipping from the lower to the upper extremities, as is the 
case in poliomyelitis. The differentiation between the ataxic forms of 
multiple neuritis or the so-called pseudo-tabes from true tabes need hardly 
be considered in the case of children, as in them true tabes is a disease of 
exceeding rarity. But it may be well to bear in mind that the symptoms of 
hereditary ataxia (Friedreich's disease), including the loss of knee - jerk, 
ataxic gait, weakness, and awkwardness in walking, may bear a superficial 
resemblance to polyneuritis ; but the gradual development of the symptoms in 



238 THE NERVOUS DISEASES OF CHILDREA. 

this hereditary disease, the occurrence in various members of the same family, 
the peculiar appearance of the face, the disturbance of speech, the mental 
peculiarities, will render the diagnosis positive enough. 

As children are subject to hysterical palsies the question of differential 
diagnosis may occasionally arise, but hysterical palsy is not characterized by 
the loss of reflexes ; nor is the ataxia, if present, like that of polyneuritis ; and, 
moreover, the anaesthesia accompanying the palsy in hysterical cases is of 
the regional order, and not in keeping with the anatomical distribution of the 
nerves. If there is any reason to suspect myelitis, the presence or absence 
of bladder and rectal symptoms, the development or non-development of bed- 
sores will weigh heavily for or against the diagnosis of cord disease. 

Trichinosis must be suspected in cases of great painfulness of the ex- 
tremities, but the other attendant symptoms (gastric disturbances) and the 
excessive swelling of the muscles will help to clear up the diagnosis. 

The recognition of the cause of multiple neuritis is an important point 
in the diagnosis of the disease. In this country the disease in children is 
developed most frequently after an acute infection, either by poisoning or 
by the toxic products of some acute infectious disease, such as diphtheria, 
variola, and typhoid fever. Next in frequency I would place the probability 
of a malarial origin, and last, the possibility of tubercular, syphilitic, or alco- 
holic infection. 

Treatment. — First of all we must determine the pri- 
mary cause of the disease, and, if possible, the deleterious 
agent must be removed or its influence arrested. If the child 
lives in a malarial district it should be taken to a place 
where there is no danger from further malarial infection. 
Tf there is any suspicion of alcoholic, or of lead, or of arseni- 
cal poisoning, the sources from which such poisons have 
emanated must be absolutely cut off. If some acute infec- 
tious disease has preceded, we can, of course, do nothing to 
prevent the natural course of the neuritis, but by improving 
the child's general health will enable it to conquer the dis- 
ease much more rapidly. For the relief of pain, which is 
by far the most annoying symptom at the beginning of the 
disease, the application of heat is the best remedy we have. 
If heat is applied at later stages of the disease, when con- 
siderable anaesthesia may be present, it is of the greatest 
importance to watch the condition of the skin, or else seri- 
ous burns and ulcerations may result. It is on this account, 
also, that the use of the actual cautery and of other forms 
of counter-irritation is not to be recommended. Prolonged 
warm baths will often help to alleviate the suffering, and 



MULTIPLE NEURITIS, 239 

will contribute largely to the comfort of the patient. If 
the pain is so intense that it cannot be relieved by these 
simple measures it will be well to give small doses of mor- 
phia, codeia, or rectal injections of chloral hydrate. 

There is no drug which has any specific action upon 
multiple neuritis. I have given the salicylates invariably, 
but am not convinced that they have done much good. 
Mercurials have been praised by some, particularly in cases 
in which the nerve-sheath is involved rather than in the 
parenchymatous form, yet I have not been able to persuade 
myself of the benefits to be derived from mercurials, even 
in many cases of multiple neuritis in the adult ; but if there 
is any good reason for the employment of mercury let it be 
given in the form of inunctions of unguentum hydrargyri 
or of a ten or twelve per cent, oleate of mercury. 

The administration of mercury, either by the mouth or 
in the form of hypodermic injections, is to be avoided in chil- 
dren, if possible. Iodide of potash or iodide of sodium has 
little or no influence upon the disease ; arsenic, which has 
gained such popularity in every form of chronic nerve dis- 
ease, is scarcely a safe remedy to use, for in small doses it 
will have no effect, and if given in large doses there is a de- 
cided danger of increasing the neuritis. We are compelled, 
therefore, in all cases of multiple neuritis in children, par- 
ticularly in those occurring after diphtheria and other acute 
infectious diseases, to resort to general tonic remedies. 
Among these none is better than cod-liver oil, and next in 
order; though far inferior to it, are iron, quinine, and 
strychnia in very small doses. In all cases of malarial 
neuritis, quinine should be given in the same doses that 
would be administered to combat other symptoms of mala- 
rial poisoning. A word of caution should be added with 
reference to the use of anodynes. In children the careless 
exhibition of morphia may result in the formation of the 
morphia habit, and morphia itself, if given in any consid- 
erable quantities, may help to intensify rather than to 
diminish the symptoms. 

If insomnia is persistent in consequence of the pain in 
the initial stages, one of the many hypnotics, such as chlor- 
alamid, trional, or veronal, in doses varying between five 



240 THE NERVOUS DISEASES OF CHILDREN. 

and fifteen grains, according- to the age of the child, should 
be given a trial ; if they do not produce sleep, codeia in 
doses of one-sixth to one-half of a grain may be adminis- 
tered. 

After the first two or three weeks of the disease the 
paralytic symptoms become rather distinct. In all those 
cases in which there is considerable paralysis with atrophy 
the use of the galvanic current, both as a sedative and as a 
means of exercising the paralyzed parts should be em- 
ployed. The faradic current will be of little service if it 
fails to produce contractions, and as it has a distinct irritat- 
ing effect upon the sensory filaments in the skin it should 
not be applied. When all pain has disappeared and the 
progress of the disease has been stayed, light massage can 
be employed to advantage ; it is specially to be recom- 
mended at the time when contractures are about to be de- 
veloped, or when the atrophy of the muscles is on the in- 
crease. If permanent contractures result from multiple 
neuritis surgical interference may be necessary, but as 
the disease generally leads to spontaneous recovery, every 
possible means of bringing this about should be employed 
before the child is given over to the surgeon ; yet I can 
see no harm in having a simple tenotomy performed if that 
will enable the child to bring its feet to the ground, or to 
walk before complete recovery from all the symptoms has 
set in. In some of the cases some simple orthopedic ap- 
paratus will help the child to learn to walk, and will enable 
it to use muscles which cannot be depended upon to sup- 
port the body ; but there is also a danger in allowing chil- 
dren to wear an apparatus too long a time, as they are very 
prone to depend entirely upon such artificial support, and, 
as a rule, lack the energy to exercise weakened muscles. 

DIPHTHERITIC PARALYSIS. 

Paralysis after diphtheria is far less frequent in this city 
than it was before the use of the Diphtheria Anti-toxin, and 
less frequent (relatively) in children than in the adult. 
In earliest infancy, say up to the age of two years, diph- 
theritic palsy is extremely rare. It bears no absolute 



MULTIPLE NEURITIS. 24 1 

relation to the severity of the diphtheritic infection. 1 have 
often seen typical diphtheritic palsy set in after an infec- 
tion so slight that it was considered to be nothing but a 
mild sore throat ; but, on the other hand, it also occurs in 
connection with severe diphtheritic infection, and is fre- 
quently associated with other complicating diseases, such 
as nephritis and endocarditis, following upon the original 
disease. The previous health of the child has no distinct 
bearing upon the development of the palsy, strong and 
weak children being affected with equal frequency. I have 
seen the paralysis developed within the first week, while the 
throat was still covered with diphtheritic membrane, but 
the majority of cases do not occur until two, three, or four 
weeks after the termination of the disease. 

Diphtheritic palsy differs from other forms of multiple 
neuritis in the order in which the various parts become 
paralyzed. The palate is generally the first, often the only, 
part affected. Nasal speech and regurgitation of liquids 
through the nose are evidences of paralysis of this part, and 
the loss of function can be determined by an inspection of 
the mouth and throat. Loss of accommodation is next most 
frequent. If the paralysis extends further, the upper and 
lower extremities are apt to be involved. At first a mere 
weakness is noticeable, associated with tingling and numb- 
ness of the parts; later on this weakness increases, and 
develops into a full-fledged paralysis, and the disturbance 
of sensation may become more marked. The ocular mus- 
cles show a peculiar predisposition to diphtheritic infec- 
tion ; the rectus externus, supplied by the sixth nerve, is 
frequently, and often singly, affected, and the oculo-motor 
nerves are also involved at times, but a complete ocular 
motor palsy in diphtheria is rare. Ptosis and paralysis of 
one or more muscles supplied by the third nerve have fre- 
quently been noticed. The optic nerve fortunately escapes, 
so that blindness is not a common result of diphtheria, un- 
less by some untoward accident the diphtheritic poison 
should cause ulceration or purulent disease of the eye, with 
subsequent loss of vision. The ciliary muscle is often par- 
alyzed ; the reaction of the pupils to light is sluggish, but 
the contraction during accommodation may be preserved, 



242 THE NERVOUS DISEASES OF CHILDREN. 

although the act of accommodation itself is carried out im- 
perfectly. 

Paralysis of the pharynx may occur, but is not frequent; 
the larynx, however, often comes in for a share of the pa- 
ralysis ; the epiglottis then fails to perform its function, and 
food may reach the larynx and cause severe fits of cough- 
ing. Hoarseness and imperfect phonation point to an in- 
volvement of the muscles of the larynx and of the vocal 
cords. This special order of paralysis is generally sym- 
metrical. 

The reflexes are diminished or lost, even when no dis- 
tinct paralysis of the adjacent parts exists or existed (Bern- 
hardt): I have had opportunity to examine several children 
whose knee-jerks were absent after diphtheria, but who 
had never presented any paralytic symptoms. The same 
condition is found occasionally after scarlatina and typhoid 
fever. 

In the limbs the change in the motor and sensory functions is very like 
that of other cases of multiple neuritis, and does not, thererefore, require 
any special mention. It is questionable whether cardiac failure coming on 
after diphtheria is due to a neuritis of the pneumogastric, or whether, as 
Leyden would have it, it is due to a degeneration of the heart muscle itself. 
The irregularities of respiration associated with the heart symptoms are so 
characteristic of vagus affections that it seems much more probable to attrib- 
ute the loss of function to disease of the nerve rather than of the muscular 
tissue. 

Some diphtheritic palsies occur which do not in any way resemble mul- 
tiple neuritis ; a hemiplegia may follow upon diphtheria, but this may be the 
result of a vascular lesion so common in connection with other infectious dis- 
eases of childhood. Cases of this character have been described by Mendel 
and Bonath. 

Paralysis of the masseters has been reported by Grant and quoted by 
Gowers. As the paralysis was permanent fourteen months after the disease 
Gowers thinks the affection due to an acute nuclear inflammation. A degen- 
eration of various cranial nerves has been observed to come on after a con' 
siderable lapse of time, following upon diphtheria, but it has seemed a little 
doubtful whether such paralysis could be traced directly to a diphtheritic poi- 
son. 

The general course of the disease leads to recovery. It 
may last from six to eight weeks ; the cases in which single 
nerves only are affected recover more rapidly than those 
with multiple nerve-lesions. The ocular nerves recover 



MULTIPLE NEURITIS. 243 

very much more quickly than the nerves of the lower ex- 
tremities do. If there is paralysis of the legs, it takes, as a 
rule, from four to six months before the symptoms disap- 
pear. The reflexes are late in returning", and in most cases 
cannot be elicited until some weeks after all other symp- 
toms have passed away. 

If death ensues during diphtheritic palsy, it is either due to some compli- 
cating disease, such as nephritis or pneumonia, or to paralysis of the heart 
or respiratory muscles. Mere exhaustion from inability to take food in cases 
of paralysis of the pharynx is an occasional but rare cause of death. 

Pathological Anatomy. — The morbid changes are 
essentially the 7 same as those found in other forms of multi- 
ple neuritis ; the inflammatory and degenerative changes in 
the nerves are in these cases probably the result of micro- 
bic poisoning, or the effect of chemical poisons formed by 
the diphtheritic micro-organisms ; but bacteriological re- 
searches have not yet yielded a very satisfactory explana- 
tion of all the changes that take place in the peripheral 
nerves. The rod-shaped bacteria, the bacilli of Klebs and 
Loeffler, have been found in the blood-vessels of the nerve- 
centres, and other micrococci have been described by Oer- 
tel as occurring in these vessels ; but further study will be 
needed to explain the origin of the inflammatory process in 
the nerves. The degeneration is found in the nerve supply- 
ing the paralyzed part ; often the entire nerve is affected, at 
times some of the filaments only. In others, the degenera- 
tion extends to the anterior or even the posterior roots. 
The myelin and nerve-filaments undergo segmentation, 
the nuclei of the sheath are increased, and granular corpus- 
cles mark the decay of nerve-tissue. The axis-cylinder 
remains intact until the destruction of the medullary sheath 
has advanced considerably, and both these parts may then 
be entirely destroyed. The interstitial tissue between the 
nerve-fibres is but little affected, thus marking the condi- 
tion as a true parenchymatous degeneration. The nerves 
supplying the palate are most frequently and most severely 
affected, but the same changes may occur in the peripheral 
nerves of the extremities in all the ocular nerves, and even 
the phrenic nerve may be similarly affected. 



244 THE NERVOUS DISEASES OF CHILDREN. 

In diphtheritic paralysis the muscular tissue is more distinctly changed 
than in other forms of paralysis due to multiple neuritis. In cases of long 
standing the muscular fibre of the palate, for instance, is found to have under- 
gone granular and fatty degeneration. In some there are signs of parenchy- 
matous as well as of interstitial inflammation, and every possible grade may 
be found between a simple inflammatory condition and complete degenera- 
tion of the muscular fibres. In cases in which the disease leads to implica- 
tion of the heart muscle, the pallor of the heart after death is the external 
evidence of complete degeneration of its muscular fibres. 

Much has been made of changes in the spinal cord in cases of diphthe- 
ritic paralysis, but these are restricted entirely to changes of the ganglion cells 
of the anterior horns, which have been found swollen, homogeneous in 
character, and with processes somewhat altered or entirely shrunken ; but it 
is doubtful whether these changes are primary or secondary, though there is 
no good reason why a poison which acts so vigorously upon the peripheral 
nerves should not invade the various tracts of the spinal cord. 

The frequent disease of the palate is probably due to the proximity of 
the nerves supplying the palate, to the diphtheritic membranes, and the eas- 
ier invasion into these nerves of the micro-organisms lodged in the tonsils, 
or in the back of the throat. And yet if we remember that a considerable 
period of time may elapse between the deposit of the diphtheritic membranes 
in the throat and the appearance of the palsy, and that the palsy may appear 
after very mild throat affections, it is not altogether easy to explain why these 
nerves should be so frequently the seat of the disturbance, unless we suppose 
that from their proximity to the original diphtheritic lesion the nerve-tissue 
has become altered and thus rendered more susceptible to the diphtheritic 
microbes, or to the microbic toxins. The poison attacks other nerves in 
the course of time ; the peroneus, the musculo-spiral, and the median are the 
most susceptible to it. 



The diagnosis of this palsy often depends upon the 
history of preceding diphtheria, or of some throat affection, 
however slight it may have been. The beginning of the 
palsy in the palate, and gradually spreading to the lower 
and upper extremities, will at once suggest the probability 
of diphtheritic paralysis. It is only in cases in which the 
original disease has been overlooked or forgotten that an 
examination of the patient some weeks after the onset of 
the trouble may, through the atrophic paralysis and the ab- 
sence of knee-jerk, suggest spinal infantile palsy ; but a 
closer examination of the patient, and the comparatively 
rapid development of the palsy, with its tendency to recov- 
ery, will place the diagnosis beyond doubt. Diphtheritic 
palsy, like other forms of paralysis in children, may con- 



MULTIPLE NEURITIS. 245 

tinue for an inordinate length of time, and may be super- 
seded by an hysterical palsy. Thus it happens not infre- 
quently, as in the daughter of a clergyman whom I examined 
many years ago, that a chronic paralysis is the outcome of 
a diphtheritic palsy which had set in a few weeks after a 
severe throat affection. 

Bear in mind the possibility of cardiac or renal compli- 
cations. The paralysis of the palate, however disagreeable 
it may be to the child, rarely leads to serious complications, 
but if the muscles of the pharynx are involved there is 
danger of food entering the respiratory tract, and of a sub- 
sequent pneumonia. A child seen recently in consultation 
was in danger of death from this cause. 

Other things being equal the earlier the paralysis appears after the initial 
disease, and the more quickly it reaches an extreme development, the more 
serious the case and the more imminent the danger to life. It is difficult to 
forecast the exact length of time which will elapse before complete recovery 
sets in, but it is safe on the whole to be guided by the same rules as in the 
case of any other peripheral nerve palsy. If the electrical examination shows 
an entire loss of response to the faradic current with an altered response to 
the galvanic current, many weeks will in all probability elapse before recov- 
ery sets in. If there are no marked changes in the electrical conditions of 
the nerves and muscles the degeneration or the inflammation of the nerve is 
very slight and the paralysis will be recovered from more quickly. 

Treatment. — The majority of cases of diphtheritic 
palsy will get well without any treatment, but it is of the 
utmost importance at all times to maintain the strength of 
the patient at par, and to be prepared for any sudden com- 
plications. If the palate is paralyzed and there is regur- 
gitation of food through the nose it will be better to feed the 
child by solids than by liquids ; if in consequence of paraly- 
sis of the pharynx and of the upper air-passages there is 
danger of food entering the respiratory tract it may be best 
to feed the child for a time per rectum, or else to use the 
feeding-tube through the nose or the mouth. In the case 
of children weakened by the diphtheritic process there is 
danger of death from exhaustion, and no time should be 
lost in employing this method of feeding rather than to 
starve the child while hoping for a spontaneous recovery. 
On the other hand, even while the rectal feeding is kept up 



246 THE NERVOUS DISEASES OF CHILDREN. 

efforts should be made every now and then to have the 
child take its food in the natural way. If in any case of 
diphtheritic palsy there is the slightest irregularity or weak- 
ness of the pulse, cardiac stimulants should be given, above 
all digitalis and small doses of strychnia. If respiratory 
weakness threatens, the prompt use of the faradic current, 
as advised by Duchenne, is quite in order. Direct excita- 
tion of the phrenic nerves by a slowly interrupted faradic 
current will have the best results. The electrical currents 
should be employed, according to the principles laid down 
in other chapters, for the treatment of paralyzed parts. 

The usual tonics — iron, quinine, arsenic, digitalis, and 
strychnia — should be given, but no one would venture to 
assert that any one of these has any special curative action 
except in the way of keeping up the general condition. 
Indeed there is no better method of treating diphtheritic 
palsy than by carefully administering food, and making 
sure that everything taken into the system will help to 
improve the nutrition of the child. * 

LEAD PARALYSIS. 

A few pages are to be devoted to this special form of paralysis, in order to 
emphasize the fact, which was proved conclusively by Dr. Putnam, that chil- 
dren are no less susceptible than adults to the effects of lead-poisoning. Dr. 
Putnam has not only shown that entire families have been poisoned by lead, 
but in a series of examinations, made for the purpose of determining the 
presence of lead in the urine of persons who were supposed to be healthy, he 
found considerable quantities in the urine of father, mother, and two of the 
children of one family, all of them being in fair health ; a third child, how- 
ever, for some unknown reason, did not exhibit any traces of the poison. 
The water drank by this family ran for some distance through lead pipes 
and was found to contain a large quantity of lead. If present in small quan- 
tities, lead is practically harmless. Knowing the varied susceptibility of 
different persons to lead, and having proved its presence in the urine of per- 
sons who exhibited no symptoms of lead-poisoning, Dr. Putnam is right in 
urging that the possibility of lead-poisoning should be taken into considera- 
tion in all obscure cases. 

As for the symptoms of lead-poisoning in children, they differ but little 
from those presented by adults, and are in entire keeping with the symptoms 

* Since the above was written, Behring's important discoveries have been made 
known. Whether diphtheritic palsy can be affected by injections of the serum remains 
to be seen ; but it is my impression that paralytic symptoms have become less frequent. 
Oppenheim does not share this view. 



MULTIPLE NEURITIS. 247 

of multiple neuritis due to other causes. The paralysis is most frequently 
developed in the extensor groups of the forearm, resulting in characteristic 
drop-wrist (Fig. 56). The muscles affected are supplied chiefly by the mus- 
culo-spiral nerve ; the supinator longus, and the extensor of the thumb usu- 
ally escaping. This escape of the supinator longus is frequently of service in 
helping to distinguish the disease from a simple traumatic musculo- spiral 
neuritis, for in the latter case all the muscles supplied by this nerve are about 
equally affected, whereas lead, like other toxic agents, such as alcohol and 
arsenic, shows a selective preference for groups of muscles that are function- 
ally, not structurally, related. In adults it is rare to find other muscles seri- 
ously affected, except those of the upper extremities, although if careful 
examination is made of such patients, a slight weakness in the lower extrem- 
ities and a diminution of the reflexes will be found to be present. I have 
myself seen two cases of lead palsy in the adult in which the entire affection 
was restricted to the lower extremities. In one case the adductor muscles of 
the thighs were the only ones affected. In children the involvement of the 
lower extremities is not exceptional, and when it does occur, is just as apt as 
not to occur earliest in the course of the disease, the upper extremities being 
affected after the lower extremities have been paretic or paralyzed. 

While the symptoms are practically identical with those of the adult, the 
diagnosis of lead paralysis will be all the more readily impressed upon the 
physician by the occurrence of other symptoms. Thus the child is apt to ex- 
hibit a general cachectic appearance ; also a peculiar ashen hue of the skin, 
severe headaches, and marked disturbances of digestion. Soltmann expressed 
the opinion some years ago that lead colic was rare in children. It is more 
correct to assert that it is difficult to distinguish lead colic from other forms 
of colic so frequent in children. Disturbance of digestion due to constipation 
is the rule, but constipation may alternate with diarrhoea and with severe 
attacks of colic ; the colic being distributed over the entire abdomen and not 
in the vicinity of the umbilicus, as is generally the case in the adult. Vomit- 
ing frequently occurs, and the vomitus may be yellowish in color. The ab- 
domen may be retracted, but in many of the cases one or all ot these symp- 
toms may be absent. 

If the lead poison attacks the brain, the tendency to convulsions will be 
very pronounced, and convulsions may possibly be the cause of death. In the 
cases reported by Karsch and Stewart (quoted by Putnam), the children died 
in convulsions, one of them with a mild form of delirium. Gowers supposes 
that epileptic attacks may occasionally originate in lead-poisoning, but Put- 
nam, whose experience in this matter is greater than that of any other author, 
is inclined to think that such a sequence of events has not been firmly proven. 

The lead line, which is of such assistance to us in diagnosticating lead- 
poisoning in the adult, is not so frequently observed in children, although in 
one epidemic forty-two cases of the sort were reported. The better condition 
of the teeth in children is unquestionably the explanation of this. 

The pathology and pathological anatomy of lead-poisoning are still sub- 
jects for discussion. That lead acts most powerfully on the peripheral nerves 
cannot be doubted, and that in the majority of cases lead paralysis is the ex- 



248 THE NERVOUS DISEASES OF CHILDREN. 

pression of a neuritis it is also safe to assume. Gombault insists that lead 
causes a periaxillary neuritis, the sheath of the nerve being more affected 
than the axis-cylinder and relatively healthy portions of nerve intervening 
between diseased parts. These changes were found in guinea-pigs that 
were not paralyzed, and Putnam suggests very correctly that such changes in 
the myelin without paralysis may account for the fact that in man the elec- 
trical reactions are very often altered before any actual paralysis appears. 
Harnack and others have suggested that lead has a direct action upon the 
muscles rather than upon the nerves, but the experimental researches of 
Stieglitz, published a few years ago, have brought the entire discussion to a 
temporary and satisfactory close by showing that lead produces changes in 
the spinal cord. Onuf, v. Monakow, and others have reported upon lead 
poliomyelitis. 

Prognosis. — The prognosis of lead-poisoning is, as a rule, entirely favor- 
able. There is more danger from the general nutritional disturbances and 
from the effect of the poison upon the brain, than from the lesion of the 
peripheral nerves and of the spinal cord, which may have given rise to the 
paralysis. If the cachectic condition is recovered from it is more than likely 
that the patient will regain considerable power in the legs in the course of a 
month or two, but the actual time that elapses before complete recovery sets 
in may vary as much in lead palsy as it does in other forms of multiple 
neuritis, and it need not be a matter of surprise if a child poisoned by lead 
shows the effects of such poisoning for six months, or even a year ; but under 
all conditions it is a source of satisfaction to be able to predict complete re- 
covery eventually. 

Treatment. — If the patient is in the acute stage of lead-poisoning the 
same measures should be employed which are advised in other cases of acute 
metallic intoxication. If the patient is in a subacute or chronic stage, an at- 
tempt should be made to eliminate the poison through the kidneys and skin. 
The iodides have become the classical remedy. It is better to clear the sys- 
tem of the lead gradually than to attempt to eliminate the poison in a very 
few days. Doses of from five to ten grains of the iodides three times a day 
will be quite sufficient for all purposes, and these should be administered in 
some alkaline water which will stimulate the kidneys to greater activity. 
Warm baths should be given every day, or every other day, in order to help 
on the same cause. 

After everything has been done to eliminate the lead, the attention should 
be directed entirely to the treatment of the paralyzed parts, and in this re- 
spect the use of active massage and electricity, in the manner so often re- 
ferred to, is advisable. The general condition of the patient will also call 
for the active exhibition of blood tonics, such as iron, arsenic, and cod-liver 
oil. 



DISEASES OF THE SPINAL CORD. 



CHAPTER XIV. 

INFANTILE SPINAL PARALYSIS — THE ESSENTIAL PARAL- 
YSIS OF CHILDREN— POLIOMYELITIS ANTERIOR ACUTA. 

Few diseases present as many interesting- problems as 
does infantile spinal paralysis. While the diagnosis of the 
disease is an easy one, there is considerable doubt as to its 
true etiology ; its pathology, which was supposed to be 
firmly established, is still under discussion, and the treat- 
ment of the disease deserves a most careful consideration. 
A full history of infantile spinal paralysis would convey a 
very fair idea of many of the important advances made in 
neurological science within the last thirty years. In no 
disease can the improved methods of studying the morbid 
anatomy of the spinal cord be put to better use, and in few 
diseases can the finer problems of electrical changes, of the 
localization of spinal functions, be studied to greater ad- 
vantage than in this acute spinal palsy. 

The clinical features of acute anterior poliomyelitis were recognized many 
years ago by the German orthopedic surgeon Heine, and his description of 
the disease still holds good, with few exceptions, to the present day. If we 
add to his description of the disease what has been discovered since his day, 
regarding the electrical reactions and the behavior of the reflexes, we shall 
have an entirely satisfactory account of all the symptoms of the disease. 
But since the days of Heine innumerable French, German, English, and 
American authors, among whom we may name Duchenne, Charcot, Marie, 
Erb, Seguin, Striimpell, Seeligmuller, Siemerling, Sinkler, Mary P.-Jacobi, 
and a host of others, have contributed to our knowledge of this disease. 
More recent authors, whose elaborate researches have added greatly to the 
proper conception of the pathology of the disease, will be referred to later on 
in connection with this special subdivision of the subject. 

Symptoms. — Acute anterior poliomyelitis comes on, in 
the vast majority of the cases, after the fashion of an acute in- 
fectious disease. Its onset is signalized by fever, vomiting, 



250 



THE NERVOUS DISEASES OF CHILDREN. 






convulsions, and even coma. All these symptoms may last 
for a few hours, or even a few days, after which time they 
gradually subside and give way to the paralysis, which is 

the most permanent 
feature of the disease. 
The paralysis is typi- 
cal of all the palsies 
which are due to a le- 
sion in the second di- 
vision of the motor 
tract.* This is equiv- 
alent to saying that 
the paralysis is of the 
flaccid order; that it 
is associated with at- 
rophy ; that the elec- 
trical reactions in the 
paralyzed parts are al- 
tered, and that the re- 
flexes in the parts af- 
fected are diminished 
or lost. The very sud- 
denness of the onset is 
extremely character- 
istic of the disease. 
There are, as a rule, 
no prodromata, and 
even in cases in which 
such prodromal symp- 
toms have been re- 
ported, it is doubtful 
whether their occur- 
rence was not a mere 
coincidence. The fe- 
ver varies between 
102 and 104 F., rarely exceeding the latter for any consid- 
erable period of time. The fever generally lasts for twenty- 
four or forty-eight hours, though in some cases in which 

* This includes the ganglion cell in the anterior horns, the anterior roots, the pe- 
ripheral nerve, and the muscle. 




Fig. 58.— Case of Infantile Spinal Palsy ; Paralysis 
and Atrophy of Left Leg chiefly. 



INFANTILE SPINAL PARALYSIS. 



251 



all the symptoms show marked severity the fever may last 
for an entire week. There is no doubt, however, that in a 
few cases of typical spinal infantile paralysis no fever what- 
ever occurs. Seguin was inclined to doubt this point, but 
the general consensus of opinion is in favor of the occur- 
rence of such an apyretic con- 
dition. The vomiting which ac- 
companies the fever, and sets 
in at a very early period of the 
disease, may resemble the cere- 
bral type, and is independent of 
any gastric disturbance. The 
convulsions are quite common 
during the first day of the dis- 
ease, are occasionally repeated 
during the first three days, some- 
times during the first week. 
They are of the order of general 
convulsions, and in this respect 
can be distinguished from the 
convulsions which occur during 
the earlier stage of acute cere- 
bral diseases. In some cases of 
poliomyelitis anterior acuta the 
convulsions are entirely absent. 
Coma is on the whole very much 
rarer than convulsions, and, if 
present, may be taken to indi- 
cate a tolerably severe form of 
the disease. The coma is not as 
profound, and not of as long duration as in many acute cer- 
ebral diseases. 

I have drawn this analogy between the acute cerebral and the acute 
spinal disease, because as a matter of fact a sharp differentiation between 
the two is often impossible in the earlier days of the disease ; and many a 
case of incipient acute anterior poliomyelitis has been diagnosticated by 
skilful physicians as the first stage of a meningitis, of cerebral hemorrhage, 
and what not. 

If these symptoms of the initial stage are not well de- 
veloped it stands to reason that but few physicians would 




Fig. 59. — Case of Acute Infantile 
Cerebral Palsy for Comparison 
with Fig. 58. 



252 THE NERVOUS DISEASES OF CHILDREN. 

be willing to make the diagnosis of a spinal infantile palsy 
with absolute certainty. The diagnosis becomes positive 
only after the recognition of the form of palsy present. 

The paralysis may possibly be present from the very 
start, but it is very often overlooked in the presence of the 
other symptoms which appear to be so much more serious. 
I have not infrequently diagnosticated this spinal paralysis 
during the first ten hours after the onset of the first symptoms. 
The paralysis is widely distributed at the start. It may in- 
volve all the four extremities, and may even involve parts 
supplied by the lower cranial nerves (bulbar paralysis) as 
observed by Medin. In some few cases disturbances of 
speech (dysarthria rather than aphasia) have been noticed. 
But the initial wide-spread paralysis rapidly diminishes 
within a few days, or within the first week, and before long 
the physician will be able to recognize the parts which 
wall remain permanently paralyzed, or at least to recognize 
those parts which will remain permanently more affected 
than others ; for it is not until after the lapse of some con- 
siderable time, say from two to four weeks, that the retro- 
gression of the paralysis ceases and an inference can be 
drawn as to the parts that will be permanently maimed. 
This retrogression of the paralysis has been insisted on by 
many authors as the most important feature of the early 
stages of the disease, but it should be added that in some 
instances the paralysis is seen to increase for a few days 
after the initial stormy symptoms have passed, then 
reaches its climax, and from this time on begins to recede 
until the few groups of muscles that are more seriously dis- 
eased give an idea of the amount of permanent injury done. 

The permanent paralysis may affect one or more parts of the body, but 
the lower extremities are much more frequently affected than the upper. In 
her article in Pepper's " System of Medicine," Mary Putnam-Jacobi, quoting - 
from Duchenne and Seeligmiiller, shows that the paralysis was most fre- 
quently distributed as follows : Left lower extremity in 34 cases ; right lower 
extremity, 40 times ; right upper extremity and left upper extremity, 23 
times ; all four extremities, 7 times ; both upper extremities, 3 times ; both 
lower extremities, 23 times ; left upper and lower extremity, twice ; right up- 
per and lower extremity, once ; right upper and left? lower extremity, 3 times ; 
muscles of trunk and abdomen, once. Of these 137 cases it will be seen that 
the vast majority involve the lower extremities. The paraplegic and mono- 



INFANTILE SPINAL PARAIYSIS. 



2 S3 



plegic form are by far the most frequent, while among monoplegias an affec- 
tion of the lower extremity is more frequent than that of the upper ; paralysis 
of an upper extremity is, however, not so rare but that it is seen often 
enough in every clinic. Special mention should be made of a paralysis of 
the deep muscles of the back (which are involved in a number of the cases) 
which gives rise to extreme 
lateral curvature of the spine. 
The hemiplegic variety is very 
rare indeed. 

The further study of the 
exact distribution of the pa- 
ralysis in poliomyelitis has 
brought out a number of in- 
teresting facts. E. Remak in 
particular has shown that the 
parts paralyzed were function- 
ally, not anatomically, related. 
In the upper extremity the im- 
munity of the supinator longus 
in spite of paralysis of all of the 
extensor muscles in the fore- 
arm brings out this peculiarity 
as well as its affection in con- 
nection with paralysis of the 
deltoid, the biceps, and the 
brachialis anticus. This latter 
form of paralysis corresponds 
with the upper-arm type. In 
the lower extremity the pero- 
neal group of muscles is more 
frequently affected than -any 
other; next in frequency the 
posterior tibial ; then the an- 
terior thigh muscles, and least 
frequently of all, the posterior 
thigh muscles. The tibialis 
anticus is generally paralyzed 
in connection with the quad- 
riceps extensor. These muscles are supplied by different nerves, but are 
associated in the extension movement of the leg during walking. The in- 
volvement of associated muscles in poliomyelitis would furnish the data for 
the study of spinal localization if the disease led more frequently to a fatal 
issue and to post-mortem examinations. 

An entire extremity, or a large group of muscles, may be permanently 
paralyzed ; in some cases the loss of function may be restricted to a single 
muscle. There is no little difficulty at times in making out the one or more 
muscles which have been permanently affected-, and in a number of cases 




Fig. 60.— Paralysis of Upper-arm, with Atrophy 
(Left Side) due to an Attack of Poliomyelitis 
in Early Childhood. 



254 THE NERVOUS DISEASES OF CHILDREN. 

which have come under my observation a difference in the electrical behavior 
has been the only safe way of determining which muscle showed a departure 
from the normal. 



In addition to this retrogressive form of paralysis, which 
may at times be widely distributed and at other times curi- 
ously limited, we have to notice the rapidly developing 
atrophy of the paralyzed muscles. I have seen a marked 
difference between a paralyzed leg and the other normal 
member within three days of the first onset of the disease, 
and it is not at all unusual to recognize the wasting of the 
limb by superficial inspection within the first week or two. 
The wasting is developed entirely in keeping with the dis- 
tribution of the paralysis, and this is so true that in one in- 
stance, which I can recall, the atrophy as well as the paral- 
ysis was evidently restricted to the upper portion of the 
tibialis anticus. In the case of the deltoid and the trapezius 
the clavicular portions only may be affected. The majority 
of the paralyzed limbs present a generally slender appear- 
ance. In some instances the difference between the two 
limbs may not amount to more than an inch or two in cir- 
cumference, in others the difference may be extreme, and it 
is not unusual to find a paralyzed extremity that is scarcely 
half the size of the normal one. The wasted paralyzed part 
makes the impression of an entirely limp, useless append- 
age, that is at the mercy of the parts whose muscles are 
still in a tolerable state of preservation. In addition to the 
reduction of the muscles the subcutaneous tissue and the 
fat often disappear, whereas in a few cases of children that 
were previously well nourished, the muscular wasting may, 
for a time, be concealed by the very considerable presence 
of adipose tissue. In addition to the reduction in circum- 
ference the atrophied parts do not grow as the normal parts 
do, whence it follows that short limbs are not infrequently 
the result of an early attack of poliomyelitis. 

The diseased member presents a few other symptoms 
which render the paralysis easy of detection. The skin 
over the paralyzed muscles often has a slightly shrivelled 
appearance ; more often still it is blue, cold, and clammy, so 
that by the mere touch of the two lower extremities the 



INFANTILE SPINAL PARALYSIS. 255 

physician can recognize the one that is paralyzed. This 
difference in the temperature of the parts is probably due to 
the improper blood-supply and to the natural shrinkage of 
blood-vessels that have no duties to perform, or else send a 
lessened supply to diminutive muscles. 

The behavior of the electrical reactions is of the utmost 
importance. With rare exceptions the paralyzed muscles 
and the nerves supplying them exhibit a complete reaction 
of degeneration. Both the nerves and the muscles fail to 
respond to the faradic current ; the nerves cannot be ex- 
cited by ordinary galvanic stimulation, and the muscle re- 
sponds in sluggish fashion, and often with an altered gal- 
vanic formula, the anodal closure contraction being equal 
to, or greater than, the cathodal closure contraction (see 
pp. 44 and 45). These changes in electrical behavior come 
on very early after the onset of the disease. From several 
examinations which I have been able to make in very early 
stages of the disease, I can assert that both the faradic and 
the galvanic response of the parts paralyzed are increased 
for the first two days, but then become rapidly diminished ; 
the nerves and muscles soon fail to respond to the faradic 
current, while the galvanic response may remain increased 
for a very long period of time ; this grows more sluggish, 
and finally there is no contraction except to very strong 
currents. It can be stated with some degree of certainty 
that those parts which continue to respond well to faradism, 
say after the lapse of a week or more, will not remain per- 
manently paralyzed, whereas the utter loss of function for a 
varying period of time of those muscles which at once 
fail to respond to the faradic current may be safely pre- 
dicted. During the later stages of the disease the return 
of the faradic response in any muscle, or a normal behavior 
during galvanic stimulation, will lead us to infer that the 
muscle, or muscles, in question mav recover their previous 
function, at least to a limited extent ; but muscles Avhich 
exhibit marked electrical changes for a considerable period 
of time have suffered a very serious injury. 

The reflexes are diminished in cases of poliomyelitis 
anterior ; but this is true only of those reflexes which are 
associated with the normal function of the paralyzed parts. 



256 THE NERVOUS DISEASES OF CHILDREN. 

Thus in a large proportion of the cases of poliomyelitis 
anterior the knee-jerk is absent ; but it is needless to say 
that it is not absent in cases of the cervical type, nor even 
in those cases in which only the posterior tibial muscles or 
the muscles of the feet are involved. 

I have in a number of instances been confronted by other physicians with 
an expression of grave doubt as to the diagnosis of poliomyelitis because of 
the presence of the knee-jerk, when a careful examination of the parts paralyzed 
proved that the paralysis was limited to the extensor muscles below the knee 
and to the posterior groups of muscles, while the quadriceps extensor and all 
the anterior thigh muscles were entirely normal. The mere presence of the 
knee-jerk under such conditions need not, therefore, militate against the cor- 
rectness of the diagnosis. 

If all the muscles of a given part are equally paralyzed 
and equally atrophied no contractures will set in, but all the 
parts will be equally limp. Thus the leg can be pushed to 
and fro by the slightest touch (Punchinello leg). Since 
locomotion and station are the results of an accurate bal- 
ancing between the extensor and flexor groups of muscles, 
it is but natural to expect that if one set of muscles is para- 
lyzed, the opposing muscles, being tolerably normal and 
having less work to do, will overact, and permanent over- 
action is expressed by contracture. As a rule these con- 
tractures do not appear until an attempt is made to use the 
affected limbs, but in a few instances — and some such have 
come under my notice — the contractures form while the 
child lies helpless on its back without any attempt what- 
ever at walking or at using the limb. It would seem from 
this that some more active cause must be at work, and it is 
probable that the inflammation which destroys the function 
of some cells may cause an irritation of neighboring cells 
and fibres, and thus produce contracture, very much as it 
it is produced in other diseases in which a lesion in the ad- 
jacent white matter acts as an irritant upon the ganglion 
cells of the anterior horns. According to Volkmann and 
others the mere weight of the body or of a limb may cause 
a deformity if the muscles surrounding a joint are paralyzed. 

The most frequent deformities are as follows : Pes equinus, or equino- 
varus ; pes valgus ; genu recurvatum and incurvatum ; permanent flexion 
(rare) ; all these deformities are evidently promoted by the attempt to use the 



INFANTILE SPINAL PARALYSIS. 2$ J 

maimed legs. Deformity of the hip is very much rarer than those affecting 
the knee or the foot. If the disease involve the upper extremities extension 
of the wrist is common in the presence of paralysis of the flexors ; clawed 
hand if the interossei are involved, and a flexion of the fingers or wrist if the 
extensors chiefly are paralyzed. The elbow-joint is rarely deformed, but is 
often entirely useless if the upper-arm muscles are paralyzed. At the shoul- 
der-joint atrophy of the deltoid may be the direct cause of a subluxation. 
The most serious deformities that occur are those due to a paralysis of the 
abdominal and deep spinal muscles. According to the extent and number of 
the muscles involved we may have a scoliosis, occasionally a kyphosis ; but 
most frequently very marked lateral curvature, particularly in the cervical re- 
gion, or a very marked lordosis in the lumbar region. From paralysis of the 
abdominal muscles the abdomen may become peculiarly distorted, and in one 
case of a child of about three, which I saw in private practice, a marked lordosis 
of the lumbar region was associated with what appeared to be an enormous 
bulging of the anterior left half of the abdomen. In accounting for these de- 
formities we must remember that in addition to the paralysis of the muscles, 
the arrested growth of the bones, and the disturbance in the general develop- 
ment of the child play a very considerable part. 

Before leaving the symptomatology of the disease it is 
important to insist on a few negative symptoms which help 
to corroborate the diagnosis in doubtful cases. It is gener- 
ally asserted that pain is entirely absent. This is true 
of the vast majority of cases, and particularly of the later 
stages of the disease ; but in one of the few cases which I 
was fortunate enough to see at an early day, pain was an ex- 
tremely prominent symptom. In such cases, therefore, the 
absence or presence of pain could not serve as a sufficient 
factor in differentiating the disease from a peripheral neu- 
ritis, or from articular rheumatism. The bladder and the 
rectum are, as a rule, not involved. This is a point of some 
importance in helping to differentiate between inflammation 
of the anterior horns and a general myelitis — a point of dif- 
ferential diagnosis that comes up frequently enough for dis- 
cussion. The entire absence of cerebral symptoms after 
those of the initial stage have passed away will help to dis- 
tinguish the spinal infantile paralysis from man)'- cases of 
cerebral palsy. 

Summarizing all these symptoms we may state that the 
diagnosis of poliomyelitis anterior may be made if the 
paralysis, however widely distributed, or however narrowly 
limited, and in whatever part of the body, comes on after 



258 THE NERVOUS DISEASES OF CHILDREN, 

an acute onset marked by fever, vomiting, and convulsions, 
and if this paralysis is associated at an early day with 
atrophy, with changes in electrical reactions, and with a loss 
of reflex activity in the paralyzed parts. 

Morbid Anatomy and Pathology. — " Poliomyelitis 
anterior due to atrophy of the ganglion cells of the anterior 
horns," has become a byword of neurological science. It 
would be fortunate indeed if such a simple statement as this 
would settle the much-disputed question of the morbid 
changes underlying the disease. 

As far back as 1863, Cornil published a case of a woman who had ac- 
quired a sudden paralysis of both legs at the age of two years, and who had 
died of carcinoma at the age of forty-nine. Cornil found the spinal cord 
much smaller than normal, and thought this due to an atrophy of the anterior 
roots and of the antero- lateral white matter. He referred to atrophic gan- 
glion cells, but did not make them responsible for the disease. In 1865 Pre- 
vost and Vulpian examined another case, also in an elderly person, who had 
died of cerebro-spinal meningitis. She had had a paralytic club-foot with 
complete atrophy of the posterior leg muscles, which was found to be due to 
an atrophy of the anterior horns of the left side, in the lumbar enlargement. 
The ganglion cells were found to be fewer in number, and the antero-lateral 
white matter was also atrophied on the same side. Very much the same 
result was obtained in another case by Clarke, but the importance of disease 
of the gray matter and of the relations of the ganglion cells of the anterior 
horns to the paralyzed parts was not fully established until the famous publi- 
cation of Charcot and Joffroy in 1870, who made the atFophy of the ganglion 
cells entirely responsible for all the symptoms of the disease. Their studies 
were based also upon the examination of the spinal cord of a woman, who 
died at the age of forty, but who had acquired an infantile paralysis at the 
age of seven years. The ganglion cells in the lumbar segment were dimin- 
ished, and were in part entirely absent. A dense sclerotic tissue marked the 
disappearance of these ganglion cells. 

Since the publication of Charcot and Joffroy's article, it 
was current belief that the chief anatomical changes in polio- 
myelitis were those involving the gray matter and its gan- 
glion cells; that the morbid process might extend through- 
out the entire length of the cord, or it might be developed 
chiefly in the cervical or lumbar enlargement. The question 
arose whether the inflammation of the anterior horns is pa- 
renchymatous in character, that is, a primary inflammation of 
the ganglion cells, or whether it is of the order of interstitial 
inflammations, in which the neuroglia is the part first affected 



INFANTILE SPINAL PARALYSIS. 259 

and the ganglion cells are destroyed later on. This dispute 
has been carried on to the present day. It was begun by 
Roger and Damaschino, and has been continued by Leyden, 
Schultze, Eisenlohr, Bramwell, Marie, Marinesco and others. 
Leyden recognized that a number of different pathological 
processes might give rise to the clinical symptoms of an 
infantile spinal palsy. He was inclined to adopt Charcot's 
theory of a parenchymatous inflammation, but in one case 
which he examined he thought the lesions somewhat similar 
to those described by Roger and Damaschino. It was evi- 
dent that this question could not be satisfactorily settled if 
the microscopical investigations were restricted to cases in 
which many years had passed between the time of onset of 
the disease and the death of the patient. 

The effort has been made to study recent cases of the 
disease. The first good opportunity was presented by a 
very extensive epidemic of poliomyelitis anterior acuta 
observed by Professor Medin, of Stockholm, and carefully 
described by his pupil John Rissler, who recorded the 
autopsies on three cases. Goldscheider, Marie, Redlich, 
and Siemerling have also published the results of post- 
mortem examinations of children who died very shortly 
after the onset of the disease. French and German authors 
have helped greatly to develop a fuller knowledge of the 
anatomical process, and their results are in direct contra- 
diction of the opinions of Charcot and Joffroy, which have 
obtained such general credence in neurology. Nearly all 
recent writers* have come to the conclusion that the entire 
gray matter is the seat of interstitial inflammation, and that 
the changes in the ganglion cells are secondary. Many of 
the authors are also inclined to regard poliomyelitis as an 
acute infectious disease, in spite of the entire absence, up 
to the present day, of proof of the microbic origin of the 
disease. They are of the opinion that the entire gray 
substance is easily affected and infected by the poison, and 
that that part of the spinal cord is most easily involved 
which has the most abundant blood-supply. 

Goldscheider's investigations, which were most carefully 
made, point to the important role played by the blood-ves- 

* Rissler and v. Kahlden upheld Charcot's views for many years. 



26o 



THE NERVOUS DISEASES OE CHILDREN. 



sels of the spinal cord. The author concludes that a condi- 
tion of irritation is present in the walls of the blood-vessels 
which leads to a dilatation of these vessels and to a prolif- 
eration of their endothelial elements. From this the morbid 
process extends to the neuroglia and produces a prolifera- 
tion of its cells. The changes in the ganglion cells are of a 




Fig. 61.— Poliomyelitis Anterior ; Part of an Acute Myelitis. Death of child (age, two 
and a half years) eight days after onset of complete palsy of legs and arms. (Sie- 
merling.) Section through lumbar segment, showing disruption of anterior gray 
matter from hemorrhage into it. B, marginal blood-vessel ; A, branch of anterior 
spinal artery. 



degenerative nature, and the changes in them, as well as in 
the nerve-fibres, are secondary and due to disease of the 
blood-vessels. Goldscheider has also shown that these de- 
generative changes occur most distinctly in the vicinity of 



INFANTILE SPINAL PARALYSIS. 26 1 

altered blood-vessels, and that the degenerated ganglion 
cells lie in vascular areas. 

The cases reported by Siemerling, while corroborating 
the views of Goldscheider and others, have a still deeper 
significance. They show that in some instances a poliomy- 
elitis anterior is merely a part of a general myelitis of both 
the gray and the white matter. The frequent restriction 
of the process to the anterior cornua must be ascribed alto- 
gether to the peculiarities of arterial blood supply. It is 




FlG. 62. — Poliomyelitis Anterior of Old Standing, showing Disappearance of Ganglion 
Ceils on Right Side, and Shrinkage of Right Half of Cord. Drawn from a section 
through lower cervical region. 

doubtful, however, whether the hemorrhage into the an- 
terior horns is often as destructive as in one of Siemerling's 
cases (Fig. 61). All these investigations prove that in in- 
fantile spinal palsy the inflammatory process is interstitial 
not parenchymatous. It may be limited to a few segments 
of the cord, or it may involve the greater part of the cord, 
and may extend to the medulla and pons. 

For the changes that occur in consequence of this early 
inflammation, and for the appearance of the cord in the later 



262 THE NERVOUS DISEASES OF CHILDREN-. 

years, we may refer safely enough to the famous publica- 
tions of Charcot and others. 

There is, first of all, a disappearance, often complete, of 
the larger ganglion cells of the anterior horns, and the few 
that are visible are altered in appearance. The nucleus has 
disappeared, the cell-body is shrunken, and the cell con- 
tours are entirely different from the normal, every trace of 
the cell processes having disappeared. But the ganglion 
cells are not the only parts that undergo changes ; the en- 
tire gray matter shrinks, and, as can be seen in the annexed 
cut, the entire gray matter in one-half of the spinal cord is 

shrunken in size, and 
^ ^ ^ the white matter of the 

same side is also very 

f P^= % mucn l ess m volume 

than that of the opposite 

half. In consequence of 

^ r* the changes going on 

UV ftJSb m t ne g ra y matter, the 

Z^P columns of Clarke dis- 

^ — ^y appear, together with 

~ , „ .. I" . 7 . ^, . other nervous struct- 

b ig. 63. — Poliomyelitis Anterior. Chronic stage ; 

section through sixth cervical segment, showing UreS. J hese Changes 

diminution of anterior gray matter and of en- ^j-g unQUestionablv SeC- 
tire half of right side. (Drawn from a specimen n . , , 

kindly furnished me by Dr. Collins.) Ondary to the changes 

in the anterior horns, 
and considering the intimate relation between the anterior 
horns and the remaining part of the gray matter, as has 
been brought to light by recent anatomical investigations, 
Ave can readily understand why, in consequence of disease 
in one part of the gray matter causing destruction of nerve- 
cells, the nerve-fibres which owe their life and nutrition to 
such cells disappear as well. The anterior nerve-roots are 
smaller than the corresponding roots of the sound side. 
These changes are also, in all probability, secondary to the 
changes in the ganglion cells. 

So much for the changes to be observed in the spinal 
cord itself. The atrophied muscles also present character- 
istic conditions. The fibres are very much diminished in size, 
many of them have disappeared altogether, and the place 



INFANTILE SPINAL PARALYSIS. 263 

once occupied by the normal fibres is largely filled by adi- 
pose tissue. There is in these cases no such nuclear prolif- 
eration and no hypertrophy of fibres such as are found in 
the muscles of patients suffering from various forms of 
muscular dystrophy. But even the presence of a few 
hypertrophied fibres would not be unusual, as the stage of 
hypertrophy seems to indicate an incipient irritation which 
precedes the condition of atrophy. Marie has gone to some 
trouble to show that even the bones in cases of poliomye- 
litis undergo trophic changes. The bones are smaller than 
those of the corresponding healthy member and appear 
more rounded on cross-section than the healthy bone does. 
Theory of the Disease. — That poliomyelitis repre- 
sents an acute inflammatory condition of the anterior gray 
matter of the spinal cord is conceded on all sides, but the 
question arises what the origin of such inflammation may 
be. The only satisfactory explanation at the present day is 
to suppose that the inflammation is the result of an acute 
infection which happens to be located in the spinal cord, 
just as other acute infectious diseases show a predilection 
for other sites in the body. The microbic origin has not 
yet been satisfactorily demonstrated, but all the clinical 
facts point toward this view, and the close dependence of 
the myelitic process upon the distribution of the blood-ves- 
sels lends further color to this theory. The infectious ori- 
gin of poliomyelitis is also rendered highly probable by the 
frequent observation of the epidemic occurrence of infantile 
spinal paralysis. Such epidemics have been recorded by 
Medin and Briegleb in Europe, and by Colmer (1843) an d 
Caverly (1894) in this country. Zappert speaks of a well- 
nigh epidemic occurrence of the disease in Vienna in 1898. 

For several years past I have recorded carefully the cases of poliomyelitis 
in dispensary and private practice, and have noticed that at least 75 per 
cent, began between the months of July and October. Medin observed five 
cases in the spring of one year, and between August and November he had 
examined altogether forty-four cases of poliomyelitis which had begun during 
this period. Sinkler states that of 270 cases 213, or 78.8 per cent., were at- 
tacked in the hot months of the year, from May to September, inclusive. 

Marie supposes that an infectious embolism or thrombo- 
sis in one or more of the branches of the anterior spinal 



264 THE NERVOUS DISEASES OF CHILDREN. 

artery may be the direct cause of the attack of poliomye- 
litis. 

Marie is inclined also to infer the infectious nature of poliomyelitis from 
the close resemblance between polioencephalitis and poliomyelitis, and quotes 
approvingly the two cases of Moebius occurring in one family, in which one 
child was attacked with the form of acute cerebral palsy, and the other child 
with an acute spinal palsy. But surely this proof of the infectious theory of 
acute cerebral palsy is extremely slender, and Moebius's cases might well be 
due to a coincidence rather than to an infection, which is supposed to have 
caused a cerebral paralysis in the one, and a spinal paralysis in the other 
child. Nor can two cases occurring in the same family be considered evi- 
dence of an epidemic character of the disease. A few years ago two chil- 
dren, cousins, were brought to me, who had developed acute spinal palsy 
within two weeks of one another. The one child has remained severely 
paralyzed in both lower extremities up to the present day, the other child 
escaped with a slight paralysis of the anterior tibial group. The theory of 
infection would be a very simple one to hold in such cases, but on closer 
examination it was found that both these children had developed the symp- 
toms of their disease shortly after exposure to an extremely cold surf bath. 
The infectious theory of poliomyelitis is a very plausible one, but we cannot 
disregard other possible causes, and refrigeration, as in the two cases just 
cited, may in some instances be a powerful factor in the development of 
poliomyelitis. It seems to me that those authors make a mistake who insist 
on a single origin for such a frequent disease as poliomyelitis. In a very 
recent article (Brain, Autumn, 1904), Batten arrives at the same conclusion: 
" Such thrombosis (of the anterior spinal artery or its branches) may be pro- 
duced by many and various forms of infection and the disease is not due to 
a special specific infection." Schultze reports that he has found Weichsel- 
baum's diplococcus in the cerebro-spinal fluid obtained by lumbar puncture ; 
he thinks that occasionally poliomyelitis anterior may be a disguised form of 
cerebro-spinal meningitis. Marinesco is of the opinion that the entrance of 
micro-organisms into the anterior spinal artery produces poliomyelitis an- 
terior. Great caution must still be exercised in the interpretation of bacteri- 
ologic findings. 

Differential Diagnosis. — Meningitis can be excluded 
if there are no other signs of a meningeal process, except 
possibly coma and convulsions. In cases of meningitis these 
constitute the first of a series of many cerebral symptoms, 
such as vomiting, rigidity of the neck, headaches, cranial 
nerve affections, and the like. In poliomyelitis none of these 
symptoms appear, and the coma and convulsions last but a 
relatively short time. 

There is little difficulty in distinguishing between well- 



INFANTILE SPINAL PARALYSIS. 26$ 

developed cases of acute spinal and acute cerebral palsy of 
children, but the less pronounced types of these diseases 
cannot be easily distinguished from one another unless a 
very careful examination is made of all the accompanying 
symptoms. The mode of onset may be exactly similar in 
both ; it is, in fact, on the close resemblance between the 
two diseases in this respect that Striimpell was led to 
build up his theory of the analogy between the two. But 
aside from the symptoms of onset, the clinical features are 
almost diametrically opposed to one another. The follow- 
ing table will bring out these symptoms in the clearest pos- 
sible manner: 

Acute Spinal Palsy. Acute Cerebral Palsy. 

Onset sudden, with fever, coma, and Onset sudden, with fever, coma, and 

convulsions. Convulsions rarely convulsions. Convulsions apt to be 

repeated after first few days. repeated. 

Paralysis flaccid, associated with Paralysis spastic ; no atrophy ; asso- 

atrophy. ciated with rigidity and contrac- 
tures. 

Paralysis widely distributed, possibly Paralysis generally hemiplegic, some- 
involving all extremities, or nar- times diplegic or paraplegic. Mono- 
rOwly limited to one member, or plegia rare, 
even a single group of muscles. 

Electrical reactions altered (R. D.). Electrical reaction normal. 

Deep reflexes diminished or lost. Deep reflexes exaggerated. 

Intellect never permanently involved ; Intellect often involved ; epilepsy fre- 

no epilepsy. quent. 

Doubt may arise as to the differential diagnosis in some 
cases between poliomyelitis anterior and a peripheral (mul- 
tiple or simple) neuritis. The onset may be equally sudden 
in both, though in many cases of neuritis the onset is much 
more gradual than it is in cases of poliomyelitis. In neu- 
ritis there are, as a rule, fewer symptoms of general ner- 
vous disturbance than in poliomyelitis ; but in those forms 
of neuritis in which there is a distinct toxic infection the 
toxic poisoning may produce cerebral symptoms very 
closely akin to those met with in the earlier stages of polio- 
myelitis. The distribution of the paralysis may be the 
same in both instances, but after all it is much more likely 
to be distributed according to strict anatomical lines in 



266 THE NERVOUS DISEASES OF CHILDREN. 

neuritis than in poliomyelitis. In the latter, muscles that 
have a common function are very apt to be paralyzed to- 
gether. In former days the presence of pain along nerve- 
trunks and along nerve-branches supplying the paralyzed 
muscles was supposed to be a safe feature of differential 
diagnosis, and this holds good in a majority of cases ; but 
according to my own experience pain may be present in the 
acute stage of poliomyelitis, and if the child is too young 
to give accurate information to the physician, it is well- 
nigh impossible to determine whether the pain is a gen- 
eral one in the joints or whether it is along the distribution 
of the peripheral nerve-branches. But 1 have never seen 
pain persist for any great length of time in poliomyelitis, 
while it persists, as a rule, for days and weeks in cases of 
neuritis. The atrophy, the electrical reactions, and the re- 
flexes may be as thoroughly affected in one disease as in 
the other. The differential diagnosis can, in many in- 
stances, be made only after a close observation of the en- 
tire course the disease has taken. (See Chapter on Multi- 
ple Neuritis.) 

The various forms of progressive muscular dystrophies 
may occasionally be mistaken for poliomyelitis and vice 
versa. In cases that are seen years after the onset of 
the trouble, the initial history of the case will often be 
an important guide to diagnosis. If a case of typical mus- 
cular dystrophy is seen during the stage of atrophy, a su- 
perficial inspection of the case may suggest an old polio- 
myelitis ; but in the progressive dystrophies the atrophy 
affects an entire limb rather than single groups, or if it has 
spread to several extremities it is, as a rule, much more 
general than in cases of poliomyelitis, while in the latter 
the electrical changes are, as a rule, much more complete 
than in cases of progressive dystrophies. 

The Charcot-Marie type (Tooth, Hoffmann, myself and others) must be 
differentiated from subacute poliomyelitis. 

In the year 1900 Oppenheim described a condition observed in very young 
children which he designated as " Myatonia congenita." The symptoms 
bear close resemblance to those of poliomyelitis anterior. The legs and pos- 
sibly the arms of the child thus afflicted are limp. The trunk muscles are 
rarely affected. The deep reflexes may be diminished or lost, but the ex- 



INFANTILE SPINAL PARALYSIS. 267 

tremities are not as completely paralyzed or even as paretic as at first sight 
they would seem to be. The electrical behavior of the muscles may be more 
or less altered. There is, of course, no history of an acute onset, and the 
entire state bears the earmarks of a condition of defective development. 

I have on many occasions called attention to the muscular weakness 
associated with rickets and which may easily be confounded with poliomye- 
litis anterior. 

Prognosis. — Altogether too gloomy a prognosis is gen- 
erally given in poliomyelitis. This is based upon the 
fact that some palsy always remains, but the actual res- 
idue of palsy may be so slight that one should be care- 
ful not to depress the hopes of parents and patient. Above 
all, there is no need of predicting that the child will re- 
main a hopeless cripple for life. There is no telling at the 
outset of the disease to what extent the retrogression may 
take place ; but, of course, the more widely distributed the 
paralysis is at the beginning the larger the remaining palsy 
is apt to be, although some cases which begin in very 
stormy fashion exhibit more progress than those which be- 
gin less violently. Cases in which but a few muscles are 
paralyzed at the start often recover with very little per- 
manent injury. Very little change need be expected in 
the first few weeks of the disease, but there is reason to 
hope that those parts which show any improvement within 
the first few weeks or months after the onset of the disease 
will recover power before long, and only those parts will 
remain permanently paralyzed which after months show no 
signs of improvement. Muscles which are paralyzed, but 
which exhibit slight, or no changes of electrical reaction, 
may be regarded in a hopeful light, and, on the other hand, 
those which very soon after the onset of the palsy exhibit 
distinct reaction of degeneration, and for months afterward 
show no sign of change in this respect, are apt to be per- 
manently paralyzed. The more complete the wasting of 
the muscles, the less likely these muscles are to recover ; 
and if contractures form in the opposing groups permanent 
disability is the probable result, but even such disability 
can often be remedied by surgical procedures. 

The prognosis as regards life is, with few exceptions, en- 
tirely favorable. Cases that end fatally are apt to do so 



268 THE NERVOUS DISEASES OF CHILDREN. 

within the first few weeks of the disease. But parents are 
often most grateful for the assurance that if the child sur- 
vives, however great the paralysis may be, its mental de- 
velopment will in nowise be impaired. Hirsch, Cassirer, 
and others have shown that individuals who had poliomye- 
litis may develop amyotrophies or dystrophies. 

Treatment. — During the acute stage of an anterior 
poliomyelitis the general condition only should be treated 
and little attention need be paid to the paralysis. The child 
should be kept in a quiet room, mild antipyretic measures 
may be employed, such as small doses of phenacetin, of anti- 
pyrin, of the salicylates, and the like. In the earlier stages 
cold applications, or mild counter-irritation over that part 
of the spine which is involved in the given case, are quite in 
order ; and the attempt should be made to limit the spread 
of the inflammation by the administration of small doses of 
bromide and of ergot (a few drops of the fluid extract). 
Iodides and other drugs I have found to be utterly ineffi- 
cient. The child should during this period be carefully fed, 
and the bowels should be thorougly purged by the use of 
small but sufficient doses of calomel. After the acute stage 
is passed the paralyzed muscles demand treatment. Elec- 
tricity and massage are the most effective therapeutic 
measures. Avoid electrization of the spine ; first, because 
it is not at all certain that the electric current reaches the 
spinal cord, and secondly, because the use of strong cur- 
rents makes the child extremely restless and may do more 
harm than good. In the treatment of paralyzed muscles an 
important use of the electric current is to exercise muscles 
which are no longer subject to the will ; it supplies, in 
other words, a convenient form of gymnastics. The cur- 
rent may, in addition, improve the state of the paralyzed 
and atrophied muscles, but whether or not it increases the 
conductive powers of paralyzed nerve and muscle I am not 
willing to assert. Since we wish to make the muscles con- 
tract, the only form of current that is serviceable is that 
form to which the muscle will respond. If the reaction of 
degeneration is complete the faradic current is quite useless, 
and if the diagnostic tests have shown that the muscles re- 
spond to the anode better than to the cathode, exercise by 



INFANTILE SPINAL PARALYSIS. 269 

anodal opening or closing of the current is the only proper 
method. 

This should be done in sittings of ten to fifteen minutes once or twice a 
day, and that strength of current should be employed which is sufficient to 
produce mild contractions. Excessive contractions are not called for and 
help to increase the difficulties of application. If a muscle responds to the 
faradic current, however slightly, that current should be employed together 
with the galvanic, and it has been my habit, even in the more severely par- 
alyzed cases of poliomyelitis, to make occasional tests with the faradic cur- 
rent, first, in order to determine whether there is any sign of improvement in 
any group of muscles that have been paralyzed, and, secondly, to give such 
muscles the benefit of both currents. If the muscles react at all to both cur- 
rents, both may be employed in one and the same sitting, or given in alter- 
nate sittings.* I am a thorough believer in the good influence that massage 
has upon atrophied and palsied muscles. It helps undoubtedly to keep up 
the nutrition of such parts, and in cases in which there is an incipient ten- 
dency to contracture such tendency may be overcome by the proper use of 
massage. But this should, if possible, be intrusted to skilled manipulators 
and not to the mother of the child or to a nurse, whose " rubbings " are, as a 
rule, wholly ineffective. Passive movements are entirely in order, and some 
good results have been attained by the regular use of Swedish movements, 
such as are given by trained rubbers or by a regular system of treatment in 
a well-equipped Zander institute. 

During the chronic stages of poliomyelitis orthopedic 
measures should be employed without reserve. If the con- 
tractures have persisted for years they will not disappear 
without treatment. Tenotomies are followed by results 
fully as favorable as those in chronic ocular palsies. The 
general condition of the child and the condition of the 
paralyzed muscles improves markedly after the orthopedic 
surgeon has done his work, and such tenotomies need not 
be restricted to the tendon Achillis, but many of the other 
muscles which are in a state of contracture can as well be 
similarly treated. The application of splints according to 
the best orthopedic principles is also of great assistance to 
the child, and this, too, should not be delayed too long, for 
it is far better to have a child walk in splints than to have 
ugly deformities of the joints develop which may cripple 
him for life. Since I have recognized the truth of these 
principles I have had the satisfaction of seeing children, 

* I have seen no reason to advocate static or high tension currents in cases of 
poliomyelitis. 



270 THE NERVOUS DISEASES OF CHILDREN. 

and even very young children, walk within a few months 
after the development of a poliomyelitis, while in former 
years such children were compelled to be carried about in 
the arms of a nurse or to be wheeled about in chairs. I am 
satisfied that few cases of poliomyelitis are so severe that 
much cannot be done by the proper application of ortho- 
pedic measures. In cases in which the joints are entirely use- 
less on account of the complete atrophy of the muscles, the 
operation for arthrodesis, as suggested by Wolff and others, 
may be resorted to. In this way a leg that would other- 
wise be entirely useless may be made to subserve the func- 
tion of standing and walking, though of course the station 
and the gait of the person will always be far from normal. 
Many years ago Nicoladoni suggested the transplantation 
of tendons in these cases. The " method " was revived by 
Gluck, Lange, Vulpius, and Hoffa. Townsend and Gerster 
have operated upon a number of my patients. The princi- 
ple is a simple one : Split the tendon of the overacting 
muscle, and transplant it into the underacting; but make 
sure that the " overacting " muscles are in good condition 
and that the " underacting " muscles are not completely 
wasted. To correct the position of flaccid limbs good can 
be done by shortening of the tendons as practised by Hoffa. 
Much is to be expected of the orthopedic surgeon in this 
field. Spiller, Young, and Frazier have attempted in cases 
of poliomyelitis in which only a few muscles were paralyzed 
to improve the condition of the limb by nerve-transplanta- 
tion. (See Journal of Nervous Mental Disease, 1903, p. 369; 
also Journal of Am. Med. Ass., 1905, p. 169.) 

SUBACUTE ANTERIOR POLIOMYELITIS. 

Subacute anterior poliomyelitis is practically a mere variety of the acute 
form of the disease, and for that reason needs but little special mention. The 
entire difference between the two diseases is in the mode of onset, and ac- 
cording to our present views of the character and origin of acute spinal palsy, 
the subacute variety necessarily implies a milder form of infection than in the 
cases with a more acute and more violent beginning. The difference in the 
symptoms is also confined entirely to the difference in the manner of onset 
and the manner in which the paralysis is developed. In these cases of the 
subacute variety we find that the disease comes on very gradually. The 



INFANTILE SPINAL PARALYSIS. 2J\ 

child is ill at ease for some days or weeks, complains of weariness in walking, 
of pains in the joints and muscles. After some little while a decided paresis 
of one or more groups of muscles is observed, generally in the lower ex- 
tremities. This increases, and after a week or more a distinct paralysis is 
developed. The paralysis then increases in the affected muscles, and is apt 
to spread somewhat after the fashion of a progressive muscular atrophy from 
one group of muscles to another. There is, therefore, a progression in these 
cases at the start, but the limit is very soon reached, and from this time on a 
retrogression again sets in, though not in such a marked degree as in the 
acute cases. The fact that the retrogression occurs after a given period of 
time will dispel all fears as regards the possibility of a progressive form of 
muscular atrophy. 

The differential diagnosis between these diseases is difficult, and cases are 
confounded easily with forms of progressive muscular atrophy, particularly of 
the peroneal type, and with cases of chronic neuritis ; but the points of dif- 
ferential diagnosis which were given between these diseases and the acute 
form of anterior poliomyelitis will also help us in arriving at a correct diag- 
nosis of the subacute variety. 

Treatment. — The treatment should be conducted on the same princi- 
ples as were enunciated in the preceding discussion on acute spinal palsy. In 
those cases in which the original disease is not nearly so violent as in the 
acute form even more can be hoped from an early application of therapeutic 
measures, but it should be remembered that the natural course of the disease 
tends much more to recovery than does the acute form, so that tenotomies 
and other surgical procedures should be delayed until the disease has become 
entirely stationary, and there is no reason to think that further spontaneous 
recovery will take place. 



CHAPTER XV. 

ACUTE MYELITIS. 

Myelitis, or inflammation of the spinal cord, has been 
made to cover a multitude of diagnostic sins, both in the 
adult and in the child. If we subdivide the cases accord- 
ing to the mode of onset, we may distinguish between an 
acute, a subacute, and a chronic form ; and if the classifica- 
tion is based on the origin of the myelitis we have an idio- 
pathic, a traumatic, a tubercular, a syphilitic, and an infec- 
tious variety. As the symptoms are very much the same, 
whatever the original cause of the disease may be, it will 
be better to describe the characteristic features of acute 
myelitis, which occurs frequently enough in children to de- 
mand special study. 

Symptoms. — The symptoms in a given case will vary ac- 
cording to the site of the inflammation, and the intensity of 
the process. Its clinical features will depend upon the 
amount of cord tissue involved. In such cases more than 
in any others an accurate knowledge of the functions con- 
nected with each segment of the cord is of importance if a 
satisfactory diagnosis is to be made. In every case of com- 
plete transverse myelitis, at whatever level the area of in- 
flammation may be, motion and sensation are chiefly af- 
fected, the reflexes are disturbed, and the functions of the 
bladder and rectum are deranged. Bilateral paralysis (para- 
plegia) is the natural result of a myelitis. If the lesion is in 
the cervical portion of the cord, both upper and both lower 
extremities will be paralyzed ; the first because the very 
parts which are most intimately connected with the motion 
of the upper extremities are destroyed ; and the lower ex- 
tremities are involved because the fibres going to them are 
interrupted at the site of the lesion. The bilateral character 



ACUTE MYELITIS. 



273 



of a palsy, is after all, the one symptom which points more 
frequently to a spinal lesion than any other. 

Almost the only exceptions to this rule are those cases in which a multi- 
ple neuritis, an ascending Landry's paralysis, or double cerebral lesions 
give rise to a bilateral form of palsy. 

If the lesion is in the cervical portion of the spinal cord, 
the paralysis of the upper extremities will be of a flaccid 
order; the paralysis of the lower extremi- 
ties will be spastic in character. Anaesthesia 
will be present in the four extremities and 
in the trunk to the level of the diseased seg- 
ments; pupillary symptoms, unilateral blush- 
/^Hfi^j% * n £ (due to lesion of the sympathetic), and 
a ^s^v f paralysis of the diaphragm are present in 

some cases. If the lesion is in the lumbar 
portion of the cord, the paralysis is re- 
stricted to the lower extremities and will 
be of a flaccid character, with more or less 
atrophy. If the lesion is in the cervical or 
dorsal portion of the cord, and the. lumbar 
portion is entirely free from disease, the 
paraplegia of the lower extremities is of 
the spastic order, and the reason of this can 
be easily understood if we recall the fact 
that after a transverse lesion in any por- 
tion of the spinal cord the lateral columns 
will degenerate downward from that level 
(Fig. 64), and that such degeneration of the 
lateral columns in the presence of normal 
gray matter of the lumbar segments will 
produce a spastic form of paralysis with 
rigidities and contractures. Taking all 
cases of myelitis, the largest number affect the dorsal re- 
gion ; and in these the arms go free, as their spinal centres 
are above the site of the lesion, but the lumbar segments 
are affected by secondary degeneration. 

Sensation is impaired at a very early period of the dis- 
ease, often from the first moment of onset. In fact, in the 
cases of traumatic origin loss of power and loss of sensa- 




Fig. 64 — Seconda- 
ry Ascending and 
Descending De- 
generation follow- 
ing a Transverse 
Lesion in the Up- 
per Dorsal Cord. 
(Struempell.) 



274 THE NERVOUS DISEASES OF CHILDREN. 

tion are effected almost instantaneously, so that a person 
thus afflicted feels not only that his legs are powerless, but 
that they appear to be dead as well. Anaesthesia exists in 
all the parts that are supplied by nerves coming off below 
the site of the spinal lesion, and in the typical cases all 
forms of sensation are equally involved. Thus we gener- 
ally find that a spastic paraplegia is associated with the 
loss of touch sense, of pain sense, of thermal sense, and 
of muscular sense in the affected parts. As the anaesthesia 
is strictly dependent upon the conduction of impulses in- 
ward through the posterior roots of the cord, the extent of 
the anaesthetic area will naturally point to the upper limit 
of disease.* At the upper limit of the anaesthetic area a 
small zone of hyperaesthesia, as a rule, begins. This is 
evidence of the fact that in the segment supplying the hy- 
peraesthetic area there is a condition of irritation affecting 
the posterior spinal root-fibres, but not the chief morbid and 
destructive process. Above the hyperaesthetic area sensa- 
tion may be expected to be entirely normal. In other cases, 
instead of a distinct hyperaesthesia we have a girdle sensa- 
tion, which also marks the level between the normal and 
diseased segments. 

The state of the reflexes helps us also to determine the 
area involved. If the lesion is in the cervical region all 
the reflexes of the upper extremities are destroyed, those 
in parts below will be exaggerated. If the lesion is in the 
dorsal region the reflexes connected with these segments, 
such as the abdominal and epigastric reflexes, will be lost 
and the lower reflexes will be increased. If the lesion is in 
the lumbar region the knee-jerk will be lost and the ankle 
clonus will be absent also. In some cases in which there 
is a very narrow band of inflammation these reflexes may 
behave differently and may give one a direct clew as to the 
exact extent of spinal inflammation. Thus in one case un- 
der my observation the knee-jerk was lost, but the ankle 
clonus was present. This, taken in conjunction with a line 
of anaesthesia showing an involvement of the lower dorsal 
segment, proved that the upper lumbar region was slightly 
involved, but that the lower lumbar and sacral segments 

* The reader is advised to consult special tables on Spinal Localization. 



ACUTE MYELITIS. 2?$ 

were not directly implicated by the disease. Since dorsal 
myelitis is the most frequent form, it is also common to 
have exaggeration of the reflexes associated with spastic 
contractures of the legs. 

The electrical reactions will vary according to the seg- 
ments involved. In cases of cervical lesion the reaction of 
degeneration will be found present in many, if not all of the 
muscles of the upper extremities, at least after the lapse of 
a few days or weeks ; those in the lower extremities will 
remain unaltered. But if the lesion is in the lumbar seg- 
ments the reaction of degeneration will be present in the 
muscles supplied by nerves coming off from the diseased 
area. In cases of cervical and dorsal myelitis we may, 
therefore, expect entirely normal electrical reactions in the 
lower extremities, however thoroughly paralyzed these 
parts may be. 

A few other symptoms must be noted which are ex- 
tremely characteristic of acute myelitis and often serve to 
reveal the disease when other symptoms in the case have 
left the diagnosis in doubt. In all such cases of myelitis, in 
whatever region the lesion may be, the vesical and rectal 
reflexes are disturbed or completely abolished. The re- 
sult of this is retention of urine, with possible overflow, or 
constant dribbling of urine, and either retention of stool or 
involuntary defecation. Since the centres for these reflexes 
are in the lowest portion of the cord, and every part of the 
cord must be intact if such sensation is to be conducted to 
the higher centres, we can understand why these symptoms 
should be present, whether the lesion be in the lumbar, in 
the dorsal, or in the cervical segments. Loss of sexual 
function is hardly to be mentioned in cases of myelitis in 
children ; but priapism, due to irritation of the spinal sexual 
centre, is not infrequently present in children, and is some- 
times a very annoying symptom. Involuntary spasmodic 
twitchings occur in nearly every form of myelitis. I know 
of no symptom which is on the whole more characteristic 
of spinal lesions, and which often serves as a differential 
symptom between supposed functional and spinal paralysis. 
This spasmodic cramp is evidently due to an irritation of 
the normal ganglion cells, and the irritability is often so 



276 . THE NERVOUS DISEASES OF CHILDREN. 

great that the mildest form of sensory impulse is sufficient 
to elicit such involuntary spasms. Under these circum- 
stances the mere touch of the paralyzed part — of a toe, for 
instance — is sufficient to produce contraction of the entire 

limb. 

Trophic disturbances are exceedingly common and 
much to be feared. Bed-sores are easily developed in all 
parts on which pressure is exerted : under the shoulder- 
blades, over the sacrum, on the hips, and even over the 
internal malleoli or on the inner surfaces of the knees and 
thighs — in short, wherever parts touch, are pressed upon, 
or are pressed against each other. In the sacral region the 
constant wetting of the bed and the uncleanliness of the 
patient may increase the danger and size of bed-sores ; and 
while it is true that such bed-sores may occur even without 
any external irritation, they are greatly aggravated by the 
dribbling of urine or the involuntary evacuation of the 
bowels. These bed-sores begin, as a rule, as a mere redden- 
ing of the skin ; the epidermis is soon worn away, the 
cutis is bared, this too disappears, and gradually the ulcer 
may eat away all the subjacent parts until the bone itself is 
laid bare, provided the patient lives a sufficient period of 
time. 

Every form of acute myelitis may be accompanied by 
fever, which may vary between ioo° and 104 F. and higher. 
The fever is due to the myelitic process, or the infection, 
but is often increased, and sometimes maintained altogether, 
by the complicating conditions of myelitis. Such compli- 
cations are deep bed-sores with the absorption of putrid 
matter and the danger of phlebitis ; furthermore, the oc- 
currence of cystitis and pyelonephritis, which are not un- 
common. In cases of myelitis, in which fever suddenly 
increases, with chills, with deep remissions and sudden ex- 
acerbations, the probability of this fever being due to some 
pyaemic process is very great indeed. As soon as the dis- 
ease has passed the acute stage the fever lessens and the 
temperature will remain entirely normal until some com- 
plicating condition is established. 

Whenever a majority of the above symptoms are pres- 
ent, the diagnosis of an acute (transverse) myelitis can safely 



ACUTE MYELITIS. 2JJ 

be made. The modification of the symptoms if the mye- 
litis is not complete, or if it is subacute and chronic can be 
easily inferred from the preceding account. The question 
of greatest interest, in every case, is to determine what the 
origin of the myelitis may be. Idiopathic myelitis is, ac- 
cording to our present notions, scarcely conceivable, and 
here, as in so many other instances, it is better to say mye- 
litis from unknown cause than to assume that in some 
cases the origin is truly spontaneous. In cases occurring 
in children, without known cause, I am inclined to suspect 
slight traumatism. 

A child of eight years, a healthy, beautiful girl, while walking on a coun- 
try road, had a desire to urinate. Her mother urged her to do so on the 
road. The child hurried to one side, and in attempting to place its right 
foot on a stone not more than a few inches in height, lost its bal- 
ance and struck on the middle of the back. The child experienced pain at 
once, but was able to walk some little distance ; soon the power of its legs di- 
minished; it had to be carried, was put to bed, and within twenty-four hours 
had developed a most pronounced form of acute transverse myelitis. After 
three days anaesthesia was complete up to the umbilicus. The paralysis 
was absolute in the lower extremities. There was retention of urine and 
faeces, bed-sores were developed, and the child died from these complicating 
conditions within three weeks after the accident. 

In other cases the spinal injury is much more severe, and 
a complete destruction of the cord may be the result of in- 
jury to the spinal column. From the effect of concussion 
alone, without actual destruction of the bony parts surround- 
ing the cord, an acute myelitis may result. The myelitis 
which occurs in connection with tumors of the cord, with 
tubercular affections of the meninges, need not be separately 
considered, as it constitutes merely a part of the more seri- 
ous disease. The two forms of myelitis which are most 
common in children are those due to Pott's disease and to 
syphilitic infection ; but both these forms are so distinct 
and so important that they deserve special consideration. 

Acute myelitis does, however, occur in connection 
with other acute infectious diseases, such as typhoid, scar- 
let fever, small-pox, and the like. Some of these cases of 
supposed myelitis have probably been cases of multiple 
neuritis. Rheumatic or atmospheric influences (refrigera- 



278 THE NERVOUS DISEASES OF CHILDREN. 

tion), as a direct cause of myelitis in the child and in early 
youth, should be considered duly. A young girl, of about 
sixteen years of age, was brought to me from the South, 
with the following history: On a very warm day in early 
spring she had taken a warm bath and had sat down at an 
open window in the evening, immediately after the bath, 
with nothing but a light chemise to cover her body. She 
sat there for hours and fell soundly asleep. The next morn- 
ing she experienced considerable difficulty in the use of 
both upper extremities. Within a few days these became 
absolutely paralyzed, and the legs at the same time grew 
stiff and motionless. She has since that time, a period of 
fully sixteen years, been suffering from the effects of this 
myelitis, and even now presents an atrophic form of paral- 
ysis of the right upper extremity, with slight involvement 
of the left, and with a complete spastic paralysis of both 
lower extremities, with incontinence of urine and faeces, and 
with considerable disturbances of sensation, though the lat- 
ter have been recovered from very much more than has 
been the paralysis or the atrophy. 

The infectious origin of myelitis has been demonstrated. Streptococci 
and staphylococci have been found by Marinesco, Tooth, Russell, and others. 
The author has reported a case occurring in a young girl in association with 
multiple streptococci abscesses. Fiirstner found pneumococci in a myelitic 
focus. Strumpell lays great stress on the toxic rather than the infectious 
origin of myelitis. 

Pathology and Morbid Anatomy. — The delicate 
structure of the spinal cord seems peculiarly liable to in- 
flammatory disease. The cervical and lumbar enlargements 
of the cord are less frequently the seat of such inflammation 
than the dorsal portion. The reason of this is not easy to 
explain, although it must in all probability be sought in the 
peculiarity of the blood-supply. 

There can be little doubt that if the blood-supply of the cord is interfered 
with, necrotic softening follows as in the case of other organs. In the case 
of one form of myelitis — that due to specific disease — the relation of the my- 
elitis to disease of the blood-vessels can be clearly demonstrated. Under 
these conditions the smaller vessels are blocked by thrombi, and the result of 
this obstruction is a necrotic softening of the surrounding parts. In the cases 
of traumatic myelitis the earliest changes are due to mechanical injury of the 
part, with compression of the delicate structures of the cord by effusion of 



ACUTE MYELITIS. 279 

blood, and to the necrosis that follows such compression. It is more difficult to 
explain the exact manner in which myelitis is developed in the cases of a toxic 
character, unless we suppose that the chemically altered states of the blood 
produce coagulation of the blood and obstruction of blood-vessels, with the 
same result as in those cases in which these conditions are brought about by 
other disease of the blood-vessels themselves. The origin of myelitis from 
refrigeration and rheumatic influences in general cannot be satisfactorily ex- 
plained in this way, nor can any other plausible explanation be substituted. 
The myelitis which results from mere concussion without any visible anatom- 
ical changes must be explained on the supposition that minute changes in 
the gray and the white matter are present, such as were found in the spinal 
cords of animals experimented on by Schmauss. 

If the exact mode of origin of various forms of myelitis 
is still unknown, the morbid anatomy is no longer a matter 
of doubt. As for the macroscopical appearance of myelitis, 
the cord so diseased is generally surrounded by the hyper- 
asmic meninges, and the cord itself, if inflammation is recent, 
may appear to be congested and slightly swollen. The dis- 
tinction between the white and the gray matter is often not 
so marked as in the normal cord. There is, furthermore, a 
change in the consistence of the cord, which may be either 
slightly softer than normal or else so diffluent that as soon 
as the pia is cut open the cord flows out like creamy pus. 
This is the condition often found on post-mortem exami- 
nation ; in all probability the cord is not nearly so soft 
during life, but, like other necrosed tissue, softens consid- 
erably immediately after death. In acute myelitis minute 
hemorrhages are extremely frequent, and the altered cord 
may present the appearance of red softening. There is 
every degree of change between simple red softening and 
the condition of hemorrhagic myelitis in which the extrava- 
sation of blood, being considerable, for the time obscures 
all other changes. If the blood has been exuded for some 
time before death its color may have changed, and the con- 
dition be that of yellow softening. We also may discern a 
condition of white softening in which the white matter has 
become diffluent without any admixture of blood. If ex- 
amined microscopically the cord is found to contain ample 
evidence of inflammatory changes. Among these are dilated 
blood-vessels, with leucocytes, granules of myelin, and, fur- 
thermore, bodies well known as corpora amylacea. The 



d&& M 











■'••::, 



By ^*«^-^0' 



^: ; ;? -.;>:« 



Fig. 65. — Acute Disseminated Myelitis. F, small foci containing round cells generally 
clustered about blood vessels. van Gieson stain (after Oppenheim). 











Fig. 66. — One of the foci (F of Fig. 65) more highly magnified (after Oppenheim). 



§ l 
26 





FlG. 67. — Swollen and disintegrating cells in a case of myeliti 
2S0 



ACUTE MYELITIS. 28 1 

softened tissue also contains axis cylinders in various states 
of disintegration. 

In many cases of diffluent myelitis these changes of the individual ele- 
ments of the cord are the only ones that can be distinctly made out. But in 
the parts directly surrounding the focus of most intense inflammation further 
changes can be made out after proper hardening and staining with the various 
dyes. On such sections the blood-vessels will be found dilated, and innumer- 
able leucocytes can be seen in the vicinity of such vessels. The nuclei of the 
smaller arteries and capillaries will be found to be enorm6usly increased and 
in a state of proliferation. The sheath of the blood-vessels is very much 
distended, blocked in part by the coagulation of blood, and round blood- 
corpuscles may even be found in the adjacent tissue. 

In the gray substance the large nerve-cells are swollen and granular, 
many of these granules showing distinct evidences of degeneration. The proc- 
esses of the cells are either shrivelled up or entirely lost, the contour of the 
cells less distinctly defined, and changed from the polygonal form to spher- 
ical or oval-shaped bodies. The neuroglia of the gray matter will appear 
denser than under normal conditions. In the white substance similar 
changes will be found in the blood-vessels and in the interstitial tissue. The 
white substance often has a distinctly fibrous appearance, containing many 
spider-cells or celts of Deiters. The white nerve-fibres themselves undergo 
degeneration. The axis cylinders are irregular, swollen, and often trans- 
versely divided. In some cases the entire nerve-fibre is destroyed or disin- 
tegrated and the space once occupied by such fibres is left vacant or occupied 
by granular matter. In some cases of myelitis the nerve elements are more 
intensely affected than the interstitial tissue, while in others the changes in 
the interstitial tissue are the more prominent feature in the cross-section, and 
the nerve-tissue has evidently been destroyed secondarily. In transverse 
myelitis the changes may be distributed equally through the entire cross-sec- 
tion ; in other cases the changes may be more intense in the gray than in the 
white matter, and in some more intense in the ventral half than in the dorsal 
half. In cases of meningo-myelitis, particularly in those of traumatic or 
specific origin, the most marked changes are near the periphery. Here the 
pia will appear thickened and the morbid changes can be traced along the 
connective tissue passing from the pia into various portions of the cord. If 
the myelitis is of the disseminated order, small foci of disease may appear in 
various portions of the cross-section and in various segments of the cord, 
intervening parts maintaining a tolerably normal appearance. If the myelitic 
changes are most prominent in the vicinity of the central canal and the parts 
surrounding it, we speak of a central myelitis ; but it is rare to find such 
central myelitis without some additional symptoms of a diffuse inflammatory 
process. The nerve-roots in connection with the inflamed segments are, as 
a rule, altered, and will present appearances somewhat similar to those found 
in the white matter of the cord. The vessels are dilated, the nuclei and the 
tissues about these vessels exhibit various degrees of proliferation, the myelin 



2 g2 THE NERVOUS DISEASES OF CHILDREN. 

is disintegrated, and the axis cylinder either swollen or distorted ; but these 
degenerative changes can, as a rule, be traced only a short distance from the 
diseased cord. 

Secondary changes follow upon the area of inflammation, and the tracts 
will be affected in an upward or downward direction, according to the direc- 
tion in which they transmit impulses. Thus, after a transverse myelitis the 
lateral columns will degenerate downward throughout their entire extent, but 
an ascending degeneration will occur in the parts that transmit impulses in a 
centripetal direction. (Fig. 64.) Among those exhibiting ascending degen- 
eration are the posterior columns, the cerebellar tract, and the antero-lateral 
ascending tract. The inflammation spreads a short distance upward and 
downward by contiguity, and those parts which one would suppose to be 
subject to descending degeneration only may be affected for a short distance 
above the lesion ; but such changes are of a distinctly inflammatory charac- 
ter and altogether different from the purely secondary changes, which rarely 
offend against physiological principles. These degenerations are, as a rule, 
developed very promptly after a transverse lesion, and often continue to exist 
after the initial inflammation has pretty well disappeared. 



Whether fibres that have once been seriously altered, 
or even destroyed, can ever regain their function or can 
grow anew, is a matter of serious doubt, and yet recovery 
takes place in a fair number of cases in which absolute paral- 
ysis, with signs due to descending degeneration, had existed 
for a number of months, or sometimes for a year or more. 
We must suppose in such cases that some fibres were so 
little altered that when the inflammatory products were ab- 
sorbed they still retained the power of conduction, and it is 
more probable that such fibres may be restored to absolute 
health than that entirely new fibres can be formed within a 
nerve-sheath, or that destroyed fibres can be replaced by 
new ones. 

Differential Diagnosis. — The more or less acute on- 
set, the often sudden loss of power, the rapid spread of anaes- 
thesia, the permanency of all these symptoms, together with 
the retention of urine and fasces, and the flaccid and atro- 
phic symptoms at the level of the injured part, together 
with the spastic symptoms in the parts supplied from seg- 
ments below the level of the lesion — all these symptoms 
will leave little doubt of the diagnosis of acute myelitis. In 
addition to this the etiological factors in the case — the occur- 
rence of traumatism, a preceding syphilitic infection or pre- 



ACUTE MYELITIS. 283 

ceding- bone disease, or marked infectious influences — will 
help to corroborate the diagnosis. 

Acute myelitis may resemble hemorrhage of the cord, but in cases of hem- 
orrhage the onset is more sudden than in cases of acute inflammation, all the 
symptoms being developed within a very few minutes. There is, as a rule, 
too, much more pain than in cases of myelitis ; but hemorrhage is frequently 
enough the first stage of a myelitis, and if symptoms indicating a spread of dis- 
ease follow upon what is supposed to be an initial spinal hemorrhage, it is fair 
to conclude that a myelitis hasmorrhagica has followed upon the initial extrava- 
sation of blood. Direct injury to the spinal column may be another factor 
tending to corroborate the diagnosis of hemorrhage. 

A rapidly ascending myelitis may suggest the acute ascending (Landry's) 
paralysis, but in cases of myelitis the progress will be clearly from the level 
of the first injury, and is not apt to attack the parts in succession from below 
upward, including the trunk, as in cases of Landry's paralysis. In ascending 
myelitis, moreover, sensation is disturbed from the start, and all the trophic, 
as well atrophic, symptoms are much more characteristic of a myelitis than 
they are of Landry's paralysis. But if the myelitis begins in the lumbar por- 
tion of the cord, and gradually spreads upward, the difficulties of diagnosis 
may be extremely great. Landry's paralysis is unusually rare in children, 
whereas myelitis is relatively frequent. 

The distinction between meningitis and myelitis is not of 
great practical importance, for meningitis is rarely present 
without some involvement of the cord, and if the symptoms 
are purely meningeal, they are generally associated with 
other symptoms pointing to a wide-spread affection. A pri- 
mary spinal meningitis is a great rarity, except as a part of 
cerebro-spinal disease or after disease or injury of the spi- 
nal column. The involvement of the meninges in a given 
case will be indicated by considerable pain in loco morbi, 
and by the presence of distinct neuralgic pain along the 
nerves emanating from the diseased portion of the cord. 

The question at times arises whether a case is one of myelitis or multiple 
neuritis. In the latter symmetrically located pains are a more prominent symp- 
tom, trophic disturbances are not so marked as in myelitis, and the symptoms 
never include vesical and rectal disturbances. Moreover, we never have that 
combination of paralytic and spastic symptoms which we so frequently find 
in cases of myelitis. In cases of multiple neuritis affecting all four extremities 
the symptoms in all the extremities are entirely the same, whereas in cases of 
myelitis they would be of the flaccid order, say, in the upper extremity, and of 
spastic order in the lower extremity. 



284 THE NERVOUS DISEASES OF CHILDREN. 

A more difficult task it is to distinguish between a mye- 
litis and hysterical paralysis, and yet a careful examination 
of the patient should reveal important points of diagnosis. 
Thus, in hysterical paralysis, legs that cannot be used in 
standing or walking may be moved freely in bed ; the 
rigidity is not so marked as in cases of myelitis, and if pres- 
ent can be more easily overcome, as a rule, than in myelitis. 
Bed-sores are rarely present in hysteria, and anaesthesia, if 
present, is anomalous in distribution. The reflexes, too, are 
not so distinctly exaggerated in hysteria as they are in cases 
of myelitis. If the symptoms should point to a lumbar af- 
fection, the lack of atrophy and the persistence of the knee- 
jerks will help to differentiate the hysterical paralysis from 
a spinal paraplegia. The bladder and rectal symptoms are 
also not so marked in hysterical as in myelitic cases. The 
very suddenness of the onset in hysterical cases, the fact 
that the paraplegia is frequently due to a sudden fright, or 
a deep emotional condition, may also point to hysteria rather 
than to myelitis. But in all such cases the fact that hysteri- 
cal subjects may suffer from organic lesion should be borne 
in mind. 

Prognosis. — The prospects in cases of acute myelitis 
will vary according to the level affected. Cervical myelitis is 
naturally a more serious disease than myelitis of lower por- 
tions of the cord, for in the former an extension upward to 
the respiratory and cardiac centres constitutes one of the 
grave possibilities of the case. In these, as well as in dor- 
sal and lumbar myelitis, the danger to life arises chiefly 
from the complicating conditions, particularly from bed- 
sores, from cystitis, and pyelo-nephritis. The earlier these 
symptoms set in the graver the prospect of the case ; but 
not a few of such cases get well in spite of all compli- 
cations, and if the myelitis can be proved to be due to spe- 
cific disease, or to some other form of mild toxic infection, 
recovery is more probable than in the traumatic cases of 
myelitis or those in which the etiological factor was en- 
tirely unknown. 

The prognosis will also vary according to the intensity 
of the affection and the extent of cord involved. If all the 
symptoms are developed rapidly, then become stationary 



ACUTE MYELITIS. 285 

and show not the slightest sign of improvement for weeks 
or months, the probability of spontaneous recovery is ex- 
tremely slight ; but any improvement which sets in, either 
in the form of diminution of anaesthesia, of the disappear- 
ance of bed-sores, or of a slight gain in motion, is a hopeful 
sign of greater improvement later on. I have myself seen 
complete recovery in cases of myelitis in which the palsy 
was absolute for a period of nearly six months, with 
marked contractures and increase of the reflexes and with 
slight vesical symptoms, but I cannot recall a single case 
in which complete recovery set in if deep bed-sores de- 
veloped at an early day, and marked cystitis appeared very 
early in the disease, the only exception to this rule being 
in cases of distinct specific myelitis. 

Treatment. — In the treatment of myelitis the follow- 
ing plan should be pursued if the patient is seen during 
the acute stage. An ice-bag should be applied to the 
greater part of the spinal column; counter-irritation may 
be used, but the danger of trophic changes in the skin 
should be remembered, and such trophic changes should 
not be encouraged or started up by an excessive use of 
counterirritants. The patient should be placed absolutely 
at rest, if possible with some form of extension. His bow- 
els should be thoroughly purged, best by the use of calo- 
mel, and the bladder catheterized by careful hands and 
watched for the first signs of a cystitis. (If cystitis should 
develop, no time should be lost, even during the acute stage, 
in beginning the usual treatment for such conditions.) All 
these measures will tend, first, to make the patient more com- 
fortable, and, secondly, they will surely lessen the danger 
from complicating conditions. The diet should be of a mild, 
non-irritating kind, and the kidneys should be encouraged 
to greater activity. Under such conditions the administra- 
tion of small doses of digitalis or of the acetate of potash 
will be quite in order. Ergot was recommended years ago 
by Brown-Sequard, and may be administered with the idea 
of limiting the area of inflammation. I cannot sav that I 
have ever seen any direct results from ergot, but it seems 
to do no harm, and considering the seriousness of the dis- 
ease it may well be tried. In cases in which there is reason 



286 THE NERVOUS DISEASES OF CHILDREN. 

to think that there is much inflammatory exudation, and 
particularly in those of specific origin, the administration 
of the iodides is quite in order, or of the mercurials and 
iodides combined ; but if mercurials are exhibited it is use- 
less to give them in any other way than by inunction. 

The main objects are to prevent the serious complications 
so common in these diseases, and to give the diseased or- 
gans a fair chance of spontaneous recovery. It is of the 
utmost importance, therefore, in cases of myelitis, to keep 
the patient absolutely clean — a task not so easy in view 
of the frequent dribbling of urine and of the involuntary 
passage of faeces. The child should be placed upon a 
water-bed. Nurses should, invariably, receive instructions 
to keep the bed-linen absolutely smooth, and to promote 
this end it will be best to have the bed dusted very liberally 
with some slightly aseptic powder. In the case of female 
patients, pads should be put in place to catch the dribbling 
urine and to prevent its soaking adjacent parts ; in the 
case of boys, urinals should be used from the start. It is 
a common practice with me to order the patient's posi- 
tion in bed to be changed at least every hour, so that no 
one part is pressed upon for too great a length of time. 
If in spite of all these precautions bed-sores should form, 
these should be treated according to the best surgical prin- 
ciples. Latterly I have been in the habit of dusting the 
sores with dermatol or aristol and covering them com- 
pletely with light antiseptic dressing, shielding the dress- 
ing as well as may be from contamination by urine or 
faeces. It is better to change the dressing frequently than 
to allow any infection of the sores through uncleanliness. 
In hospitals or among the poorer classes less expensive sub- 
stances, such as bismuth, may be used ; and if cystitis has 
been set up, the bladder should be washed several times a 
week with some weak antiseptic solution. 

After the symptoms of the acute stage have been suc- 
cessfully treated, and the patient has passed into a more 
or less chronic condition, the question arises as to the 
proper treatment of this latter stage of the disease. I am 
in favor of making the attempt again and again of affecting 
the focus of inflammation by the administration of the 



ACUTE MYELITIS. 287 

iodides. In nine cases out of ten this will be unavailing, 
and yet there is no good reason why the effort should not 
be made. But the possibility of recovery should be well 
weighed in the scales as compared with the gastric disturb- 
ance which these drugs so often excite. The nutrition of 
the child must be maintained at all odds, and it is far better 
to abandon the iodides than to permanently impair the as- 
similation of food. If the iodides cannot be given, inunc- 
tions of the oleate of mercury or of the usual mercurial 
ointment should be substituted for the iodides. Counter- 
irritation may be attempted, either by the cautery, by blis- 
tering, or by mere cupping ; but little direct good is to be 
expected from these remedies. The child should be kept 
absolutely at rest, and the spinal column should be disturbed 
as little as possible. Electricity applied to the spinal cord 
is of very doubtful utility, but there is all the more reason 
for using it in the treatment of the paralyzed parts. It 
does excellent service here as a form of exercise for the 
maimed limbs. Massage has very much the same, and, I be- 
lieve, a better, effect than electricity. It tends, in addition, 
to maintain the nutrition of the parts, and to overcome the 
tendency to contractures, which so frequently give rise 
to the most disagreeable symptoms in these cases ; but nei- 
ther electricity nor massage should be pushed if the invol- 
untary spasmodic contractions become more frequent, as 
they often do in consequence of these measures. These 
contractions are not harmful, but are extremely irritating to 
the patient, and if inordinately increased may disturb his 
rest, and thus interfere with the general nutrition. 

As soon as the child has sufficiently recovered, it is of 
the greatest importance to give it all the fresh air possible, 
and, if necessary, to provide it with a wheeled chair, so that 
it can be given its regular outing. Its diet must be care- 
fully looked to, and all unnecessary excitement should be 
avoided. Tonic measures may be employed ; and if the 
child is in the charge of a competent person give lukewarm 
baths, followed by cool or cold douches of the spine, and 
let this be done before the massage is given. Iron, quinine, 
or arsenic may be administered for their general tonic ef- 
fect. Strychnine may be given in ver} T small doses in those 



288 THE NERVOUS DISEASES OF CHILDREN. 

cases in which there is reason to think that the substance 
of the cord has not been absolutely destroyed, and that the 
function of the diseased parts could be increased by the use 
of this drug. I know that it is an extremely popular drug- 
in these diseases with all physicians who are not specialists, 
but I have found it to be a double-edged weapon. If it in- 
creases nerve conduction for a time, such improvement is 
very apt to be followed by a further diminution of func- 
tion, and in other cases again it produces annoying mus- 
cular contractions, which are as disagreeable as those that 
result from an excessively strong electrical current. In 
these cases, too, the physician who watches his patient 
carefully, and does not attempt to do too much, will suc- 
ceed far better than he who is continually meddlesome and 
ever anxious to change treatment. 

INJURIES OF THE SPINAL CORD. 

The direct results of jumping, of falls, and of injury due to falling weights 
are, on account of the more delicate nature of the spinal vertebrae, more seri- 
ous in childhood than in later years. A fall downstairs, or a simple fall out of 
bed, or a mild blow inflicted by another child, may be a sufficient cause to start 
the symptoms pointing to actual injury of the spinal cord. 

The symptoms of spinal-cord injury may be extremely varied. I do 
not propose now to discuss those cases in which traumatism is the remote 
and questionable cause of a subacute or chronic form of spinal disease de- 
veloping months, or even years, after an accident ; but I wish particularly to 
direct attention to those cases in which the spinal injury is followed immedi- 
ately, or within a period of a few weeks, by symptoms which point to some 
traumatism. In the severest form of injury to the cord there is immediate 
paralysis both of the motor and sensory functions. Inasmuch as the injury 
more frequently involves the dorsal and lumbar portions, a spastic paraplegia 
with anaesthesia of the lower extremities, with loss of vesical and rectal con- 
trol, constitute the chief symptoms which may be developed within a few 
minutes or within a few hours after the injury. 

If examined more in detail we can determine by the character of the 
symptoms, first, the exact portion of the cord involved by the injury, and sec- 
ondly, the amount of injury done at any level. The question of the exact 
extent of the injury up and down the cord is determined by the parts para- 
lyzed, and more particularly by the extent of the anaesthesia. These cases are 
indeed well calculated to illustrate the principles of spinal localization. In 
cases of cervical lesion the upper as well as the lower extremities are in- 
volved. In the upper extremities the paralysis is of an atrophic order, while 
spastic paralysis is present in the lower extremities. The anaesthesia in- 



ACUTE MYELITIS. 



289 



volves both the upper and lower extremities as well as the trunk, to or 
from a level supplied by the nerves coming off from the injured segment. 
In cases of cervical injury the sympathetic may be involved. In cases of in- 
jury to the dorsal region, which are the most frequent, the arms are not af- 
fected, the lower extremities are in a condition of spastic paralysis, the 
bladder and rectum may be involved, and the anaesthesia extends from be- 
low upward to a level corresponding to the segment or segments injured. 
The band of hyperaesthesia, or the upper level of the anaesthesia, will cor- 
respond to the upper limit of injury. If the 
injury has been done to the lumbar enlarge- 
ment, the paralysis of the legs is of a flaccid 
order, the reflexes are diminished or lost, the 
vesical and rectal reflexes lost, and the areas 
of'anaesthesia will vary according to the seg- 
ments involved. Injuries to the cauda equina 
are of particular interest because of the pecul- 
iar character of the anaesthesia, upon which 
alone an accurate diagnosis can be based.* 

The exact study of the symptoms will 
often help us to determine the upper as well 
as the lower limits of injury. Thus in one 
case which I have had occasion to observe, 
there was spastic paralysis with slight dimi- 
nution of the knee-jerks, but presence of 
ankle clonus. There was spasmodic twitch- 
ing of the legs, loss of vesical and rectal 
reflexes, as well as a tendency to bed-sores. 
The difference in the behavior between the 
knee-jerks and the ankle clonus proved con- 
clusively that the injury had slightly involved 
the upper lumbar segments, chiefly the lower 
dorsal region, but that every part of the cord 
below the upper lumbar segment had escaped 
injury, or else the ankle clonus would surely 
not have been present. In determining the 

extent of the cross-section involved, a very important question, and one which 
helps us to decide whether a complete crush has taken place or not, we must 
keep the physiology of the cord in mind, and endeavor to make out whether 
the anterior horns as well as the lateral or the posterior columns have been 
involved. Thus, in a case in which the lesion was in the lower dorsal region, 
and in which there was complete anaesthesia with marked spastic paralysis, I 
found that the muscles of the back showed, after a lapse of weeks, neither a 
tendency to atrophy nor to changes in electrical reactions. I argued from 
this, and correctly too, as the event proved, that the chief injury was done 
to the posterior and lateral portions of the cord, and that the ventral portions 
had entirely escaped. 

* See the paper by Starr in American Journal of the Medical Sciences, July, 1892. 




Fig. 68.— Sketch of Section of 
Spine in a Case of Fracture 
Dislocation of the Seventh 
Cervical Vertebra. (After 
Thorburn. ) 



29O THE NERVOUS DISEASES OF CHILDREN. 

Another aid to accurate diagnosis of the lesion will be found in the sen- 
sitiveness to pressure over the spinal column at the seat of injury. The 
parts that are sensitive should correspond to the segment of the cord which 
an examination of the patient has shown to be diseased, and before coming 
to any definite conclusion the physician should remember the relation of the 
external parts to the segments of the cord. If these two sets of facts do not 
accurately correspond, the preference should, to my mind, be given to the 
seat of injury as determined by the study of the paralysis and the anaes- 
thesia ; but if the level determined in this way is not far distant from the seat 
of pain on pressure, both should be included within the area to be operated 
upon. 

The course of the disease will depend largely upon the region affected 
and upon the extent of injury done. Injuries to the cervical region are, on the 
whole, more serious than those to the dorsal and lumbar portions of the 
spinal cord. Injury to the lumbar spine is generally followed by more serious 
symptoms than is the case after dorsal injury. If the initial symptoms in- 
dicate a comparatively slight lesion at any level, the progress of the disease is 
apt to be more favorable than if a complete or nearly complete crush of the 
cord has occurred. If the symptoms show a tendency to improvement after 
a few weeks, or after a month or more, the possibility of complete recovery 
may be considered ; but if they remain stationary for a long period without 
the slightest indication of improvement, actual recovery is rare, unless re- 
lieved by operation. 

The danger to life is greater in the cervical cases, in which the proximity to 
the vital parts is of much importance ; and in severe cases of dorsal or lumbar 
injury the complicating conditions, such as cystitis and bed-sores, may bring 
about a rapidly fatal issue. After an initial injury that is relatively slight, de- 
generation may set in, which will be characterized by the onset of rigidity and 
contractures, and from the onset of these symptoms the prognosis as regards 
complete recovery may become very much graver. 

Pathology. — The actual anatomical changes in traumatic lesions of the 
spinal cord may vary greatly. Even without injury to the vertebral column 
hemorrhage may occur from the effect of the shock, and this may be either 
epidural or subdural. The probability of the seat of the hemorrhage will have 
to be argued from the general character of the symptoms. In persons whose 
arteries are fragile, hemorrhage is much more likely than in persons whose 
vascular system is entirely normal. Persons with syphilitic disease will, 
therefore, be much more liable to traumatic hemorrhage than those not so 
affected. If the hemorrhage is considerable, whatever its location may be, 
the cord will suffer from compression and may undergo softening unless the 
blood that is exuded is rapidly absorbed. In all cases, inflammatory prod- 
ucts, which may be tinged by blood, will be found at the seat of injury. At 
times, in spite of a sudden paralysis developed immediately after an injury, 
no tangible lesion can be discovered at the time of operation or on the post- 
mortem table. We must then suppose either that the evidences of the initial 
lesion have disappeared, or that the traumatism has resulted in functional 
changes, or in such, at least, as are beyond the discovery by our present 



ACUTE MYELITIS. 29 1 

methods. If examined months or years after the initial injury, the cord may 
present nothing but the ordinary symptoms of chronic myelitis with consider- 
able shrinkage and wasting of the entire substance of the cord. Such wast- 
ing may be at times more marked in the white columns, at other times more 
distinct in the gray matter. In addition to the local changes found, the cord, 
if examined carefully, will reveal ascending and descending degenerations in 
accordance with the intensity of the process at any given level. I have seen 
several cases of spinal injury with severe spinal symptoms in which at the 
time of the operation no tangible changes were found in the cord, but the 
cord was evidently compressed by inflammatory exudations that had collected 
between the bone and the dura. Adhesions also form under these conditions 
between the dura and the surrounding parts. The impairment of function is, 
therefore, due to extra-spinal conditions. The breaking up of such adhesions 
and the removal of such inflammatory products are followed by improvement 
in the condition of the patient, if such removal is effected within a relatively 
short period of time after the accident. 

Treatment. — In cases of serious injury to the spinal cord, absolute 
rest is essential. The case may be treated in every respect for the first few 
days as though it were a case of non-traumatic myelitis, that is, by applica- 
tion of cold to the spine and by extension. As soon as the condition of the 
patient will permit, a careful examination should be made in order to de- 
termine the amount of injury done. The details of the treatment will not 
vary from that advised in cases of myelitis. The old habit of using the 
actual cautery, of blistering, and the like, is not to be recommended, for 
little good can follow it. As w r as said in the case of myelitis, the danger of 
trophic disturbances in the skin is great enough without such additional en- 
couragement. The most important question that arises is whether anything 
can be done for the patient by surgical means. There is still much hesita- 
tion on the part of physicians and surgeons in this respect. 

If the patient is in good general condition, and is able to stand the shock 
of the operation, the advisability of such surgical interference should be con- 
sidered purely upon the merits of the case, and if the evidence points to the 
fact that injured bone is pressing upon the cord, or that severe hemorrhage 
has occurred and that there is danger of permanent harm to the cord, the sur- 
geon should be permitted to expose the injured region. This can be done 
with considerable impunity by competent surgeons at the present day, and, 
as I have said, should be done as early as practicable after the injury. The 
cases in which surgical interference is useless are those in which all the 
symptoms indicate absolute crush of the cord, or in which the rapid devel- 
opment of all the symptoms points to a probably fatal issue ; or those in 
which so long a period of time has elapsed since the injury that there is no 
good reason to believe that the conditions can be relieved by operation. I 
have advised operations upon the spinal column in four cases, two of which 
were in children under the age of fifteen. The results were satisfactory, al- 
though neither one attained complete recovery ; but I attribute this to the 
fact that the cases were sent to me at too late a period after the accident. 
The operations are not nearly so dangerous as those upon the brain, and 



292 THE NERVOUS DISEASES OF CHILDREN. 

much encouragement may be derived from the statistics of various surgeons 
who have not lost a single case operated upon for serious spinal injuries. If 
for some reason or other the operation has not been performed and the patient 
is left with a chronic spinal disease, the attempt may be made to benefit the 
patient by the administration of the iodides ; and the crippled condition of the 
extremities may be somewhat improved by ordinary surgical and orthopedic 
measures, such as have been frequently referred to in the discussion of mye- 
litis and other forms of palsy. 



CHAPTER XVI. 

SYPHILIS OF THE SPINAL CORD: SPECIFIC MYELITIS AND 
MENINGO-MYELITIS. 

In the adult, syphilis of the spinal cord can now be recog- 
nized by a definite combination of symptoms. This is due 
to the work of Erb, Gowers, Marie, Oppenheim, Siemerling, 
Hoffmann, the author, Nonne, and others. In the child 
syphilitic disease of the spinal cord is not nearly so frequent 
as in the adult, but I do not hesitate to devote a special sec- 
tion to this subject, both because I think such cases can be 
easily overlooked and because they present a number of 
points in differential diagnosis which are of the greatest 
interest and which must be carefully considered if grave 
errors in diagnosis are to be avoided. 

Symptoms. — In former days it was customary to make 
the diagnosis of syphilis of the spinal cord if the symptoms 
pointing to spinal lesion were irregular and would not fit 
into any of the ordinary types of spinal disease. As a mat- 
ter of fact the nature of the morbid process underlying 
these syphilitic spinal diseases is such that irregularities in 
distribution, and in the development of the symptoms, are 
very apt to occur, and we should be able to recognize the 
symptoms of syphilitic disease whether or not the patient 
reveals, or we can prove, previous syphilitic infection. 

In all but a few of the cases the onset of the disease is 
gradual. By degrees the legs or the arms, or both the 
lower and the upper extremities, which have shown some 
weakness, become paralyzed. One leg, or one arm, is at 
times more paralyzed than the other. This paralysis may 
be of the atrophic kind, but is much more apt to be of a 
spastic order. It is often associated with intense pain (par- 



294 THE NERVOUS DISEASES OF CHILDREN. 

aplegia dolorosa), or with anaesthesia — the anaesthesia and 
paralysis may be crossed (Brown-Sequard type). The re- 
flexes are generally increased, rarely absent. If the specific 
process is situated in the cervical segments there are atrophic 
paralysis with loss of reflexes in the upper extremities, spas- 
tic paralysis with rigidities and contractures in the lower 
extremities. The vesical and rectal reflexes may be inter- 
fered with. If the dorsal or lumbar segments are involved, 
the symptoms will closely resemble those following upon 
myelitis of the respective segments ; trophic disturbances 
may occur ; bed-sores may develop ; in short, we may have 
all the symptoms of a wide-spread spinal affection. It is evi- 
dent that if we wish to distinguish between specific disease 
of the cord and the various forms of acute or chronic my- 
elitis we must look for some distinct points of differential 
diagnosis. 

Erb, referring to be sure to the conditions in the adult, 
has established a type of spinal-cord disease which he pro- 
posed to call syphilitic spinal paralysis. This special type 
bears the following characteristics : First, the usual symp- 
toms of spastic paraplegia, with its peculiar gait, carriage, 
and movements ; second, marked exaggeration of the deep 
reflexes ; third, muscular contractures, which are slight as 
compared with the exaggeration of the reflexes ; fourth, 
involvement of the bladder ; fifth, a slight yet distinct dis- 
turbance of sensation ; sixth, gradual onset of the disease ; 
seventh, a decided tendency to improvement. There is no 
doubt that this type of spinal disease, so well characterized 
by Erb, does occur. The same series of symptoms has been 
recognized by Rumpf, and since Erb's publication cases of 
this description occurring in children have been observed 
by Friedmann and by myself. But I have taken some pains 
to prove that there are other types, quite as frequent as this 
one, and that it is a great mistake to hesitate in making the 
diagnosis of spinal-cord syphilis unless the symptoms of 
Erb's type are present. It is the author's conviction that, 
if we wish to make a positive diagnosis of syphilis of the 
spinal cord, we should pay attention to the following points, 
and not exclusively to those presented by Erb as character- 
istic of the special type he has described. 



SYPHILIS OF THE SPINAL CORD. 295 

First, the most striking feature of syphilis of the spinal 
cord is the unusual distribution of the disease over the 
greater portion of the cord, involving, as it often does, the 
cervical and dorsal, as well as the lumbar enlargements. 
Second, the slight intensity of the morbid process at one level 
as compared with the extensive area involved, as evidenced 
by the preservation of some of the functions of the cord 
with complete loss of others. Third, the rapid dwindling 
of some of the symptoms and the very chronic persistence 
of others. Thus in some of my cases the anaesthesia lasted 
but a very short time, while the paralysis was recovered 
from with extreme slowness. Fourth, the very frequent 
history of other symptoms pointing to specific disease in 
the same or distant parts of the central nervous system. 



To emphasize these views let me state that I should be inclined to suspect 
specific disease of the cord if the patient presents symptoms of paralysis, 
whether they be of the spastic or flaccid character, and whether the contract- 
ures be slight or not, provided he furnish evidence of a morbid process affect- 
ing a very large part of the cord, and yet showing a relatively slight intensity 
at any given level of the cord. He may, for instance, exhibit the symptoms of 
extreme paralysis, spastic or atrophic, with partial or slight anaesthesia of the 
parts paralyzed, with little or no involvement of the bladder ; or, as often hap- 
pens, he may present traces of specific disease in other parts of the central 
nervous system. The chief difference, according to this, between the ordinary 
forms of myelitis and the specific diseases of the cord can be understood if 
we remember that the symptoms of an acute or subacute myelitis prove that 
the entire cross-section of the cord is affected almost simultaneously and to 
an equal degree, whence it follows that in such cases severe paralysis is likely 
to be associated with severe anaesthesia, with marked contractures, with abso- 
lute loss of vesical and rectal control, with serious trophic disturbances, and 
so on ; whereas, in the cases of spinal syphilis the morbid process invades the 
cross-section of the spinal cord partially and slowly. We may, therefore, 
find symptoms which point to a very marked affection of one or more of the 
systems of the spinal cord and to relative immunity from disease of the gray 
matter or other portions of the cord. Thus we may have extreme paralysis, 
but only slight anaesthesia ; or extreme loss of power, with relatively slight 
rigidity, as Erb pointed out. Furthermore, in the ordinary cases of myelitis, 
the symptoms point to a certain portion of the spinal cord at which the dis- 
ease is most intense, while in cases of syphilis of the spinal cord the clinical 
symptoms show that the disease involves a very large portion, if not the en- 
tire spinal cord, yet affects each single segment but relatively little. A still 
further aid to differential diagnosis is the very frequent involvement of the 
brain, at the same time that the majority of the symptoms point to disease of 



296 THE NERVOUS DISEASES OF CHILDREN. 

the spinal cord, or if these two sets of symptoms do not set in simultaneously, 
we frequently have in a patient who presents symptoms of a specific myelitis 
the history of a preceding illness in which the symptoms were. of a cerebral 
rather than of a spinal character. (Unequal ocular, and, above all, unequal 
pupillary symptoms, are very common symptoms of cerebral syphilis.) The 
rapid and often unexpected recovery, as well as the relapses, help also to dis- 
tinguish these cases from the usual forms of myelitis. 

In view of the rarity of these diseases, or possibly of the 
failure to recognize them when they do occur, I wish to 
give a few typical cases. The first one I take from Fried- 
mann's article on relapsing, probably specific spastic, spinal 
paralysis in childhood. 

A boy, five years of age at the time of examination. Nine months before 
the birth of this child the mother miscarried with twins at the end of the sec- 
ond month of pregnancy. The birth of the boy was entirely normal, but 
the head was said to have been very large. Relative reduction of the size of 
the head in the next few months. Four weeks after birth a skin eruption 
appeared which covered the entire body ; it was vesicular at first ; later 
on it ulcerated, and terminated in desquamation. The child began to ex- 
hibit normal mental development, learned to talk at the end of one year, 
and began to teeth at the age of six months. At the age of three months 
there was distinct difficulty in moving the arms and legs. Three months 
later the right arm could be moved, and at the age of one and a quarter years 
all four extremities appeared to be entirely normal. In the second year, the 
child having learned to walk well, paralysis again gradually developed in the 
left arm, and disappeared once more after six weeks. From the second to the 
fourth year the boy was healthy, and passed through measles and diphtheria 
without any disagreeable sequelae. In the fourth year he complained much 
of headache, particularly in the occipital region, and gradually his gait became 
weaker and weaker, and he began to drag the right leg, frequently falling in 
the attempt to walk. There was slight difficulty in micturition and the legs 
were somewhat rigid. In this attack the arms were entirely free, and there 
was no history of spasms or convulsions at any time. The patient was in 
this condition when examined by Friedmann. The only other points of in- 
terest in the case were the very bad condition of the teeth, slight increase of 
the reflexes, and entirely normal sensation. 

More recently J. Hoffmann has reported the case of a 
boy, who, at the age of twelve years, developed a typical 
spastic paraplegia, evidently due to hereditary syphilis, 
manifest signs of which appeared in the first years of life. 

The following case, observed by myself, presents many 
similar features : 



SYPHILIS OF THE SPINAL CORD. 2()J 

E. S , a girl, aged six years, born in this country, of German parents, 

was brought to my clinic in June, 1893. The mother gives a history of pro- 
tracted labor, but child was entirely normal ; began to cut teeth at six months, 
to stand and walk at the age of one year, and learned to talk well before she 
was two years old. 

The child had been perfectly well, with exception of mild attacks of 
whooping-cough and measles. At the age of five years the mother noticed 
that the child began to walk in a peculiarly stiff manner, and that its mental 
development was somewhat retarded. On examination we noted spastic par- 
alytic gait ; spastic paraplegia of lower extremities, more marked on left side ; 
left upper extremity slightly paretic and rigid ; both knee-jerks exaggerated ; 
triceps and wrist reflexes lively on left side. Pupils unequal ; left pupil dilated 
and does not react to light ; right pupil reacts sluggishly to light ; both pupils 
react sluggishly during accommodation. No sensory symptoms. 

The suspicion of syphilitic disease was strengthened by an examination of 
the mother. She has had two miscarriages ; three children died in early life. 
Five years ago (at the age of thirty years) she had left hemiplegia ; no loss of 
consciousness ; recovered in a few weeks ; has distinct " rheumatic " attacks ; 
gets dizzy while washing her face in the morning ; her pupils are unequal ; 
no reaction to light or during accommodation ; knee-jerks absent ; slight 
Romberg symptom ; no bladder trouble ; delayed sensory perception in the 
lower extremities. 

Thus we have a tabic, if not a purely syphilitic affection in the mother, and 
spinal syphilis in the child. 



Differential Diagnosis. — It seemed to me best to in- 
troduce most of the salient points of differential diagnosis 
in the description of the symptoms of the disease, and the 
comparison with myelitis was inevitable. On the distinc- 
tion between myelitis of the ordinary types and specific 
disease of the spinal cord, I need say nothing more. Syph- 
ilis of the spinal cord might be confounded with a spastic 
infantile palsy, particularly with a spastic diplegia and 
paraplegia resulting from meningeal hemorrhage occurring 
during the period of labor ; but in these latter cases the 
trouble can be traced distinctly to the earliest period of 
life, and there is never any history of relapses, and rarely of 
any marked improvement followed by relapse. Moreover, 
in the typical cerebral palsies the contractures are apt to 
be more extreme, and defective cerebral development is 
much more frequent than in the syphilitic cases coming on 
subsequently to the birth of the child. An acute infantile 
cerebral palsy might possibly simulate specific disease, 



298 THE NERVOUS DISEASES OF CHILDREN. 

were it not for the far greater frequency of the hemiplegic 
form of paralysis in these cases than in the purely syphi- 
litic types of disease. Special difficulties might, however, 
arise in cases in which the acquired infantile hemiplegia 
would be proved to be due to syphilitic disease of the ar- 
teries, and under such conditions a combination of spinal 
syphilitic disease with cerebral disease would not be im- 
possible, although I have not yet come across a case of this 
description. Spinal syphilis in a child should not be con- 




FiG. 69.— Case of Multiple Cerebro-spinal Syphilis. Section through pons showing 
gummatous formation {g) in ventral portion, with considerable destruction of tissue. 
Weigert's haemotoxylin stain ; low power.* 

founded with hereditary spastic paralysis ; possibly the 
latter may be developed on a specific basis. 

Morbid Anatomy. — The anatomical changes in the 
spinal cord, due to syphilis, may be quite as varied in the 
child as in the adult. It is well known that syphilis is apt 
to cause disease of the blood-vessels. Obliterative endoar- 
teritis, with subsequent softening of the area supplied by 
the diseased vessel, is perhaps the best known anatomical 
process directly attributable to syphilis ; but a general ar- 

* Figs. 69-71 are reproduced by the courtesy of the editor of the New York Medi- 
cal Journal. 



SYPHILIS OF THE SPINAL CORD. 299 

teritis is quite as frequent as an inflammation of the endo- 
thelium alone. Bruce has directed attention to changes in 
the adventitia (nodose periarteritis); moreover, veins are 
subject to syphilitic changes quite as often as the arteries 
are (phlebitis obliterans, GreifT). All these vascular changes 
are much more pronounced in the pial covering than in the 
substance of the spinal cord. 

The investigations of other authors, as well as my own, 




Fig. 70.— Section from Ventral Surface of Medulla Oblongata (high power), show- 
ing Infiltration of Pia and Substance of Medulla (a) and Typical Syphilitic Arteritis ; 
Marked Thickening of, and cellular proliferation in Intima (i) ; Narrowing of the 
Lumen ; Cellular Infiltration of Adventitia (£). 



have shown that syphilis of the spinal cord is more often 
associated with a subacute or chronic meningitis, or me- 
ningo-myelitis, than with any other process. The disease 
starts, as a rule, in the pia, and subsequently invades the 
spinal cord. There can be little doubt, however, that the 
changes may in some instances be developed in the reverse 
order. 

In the gross specimen we find the pia thickened and 



3oo 



THE NERVOUS DISEASES OF CHILDREN. 



often covered by a thick gelatinous substance. On micro- 
scopical examination this thickening is seen to be due to a 
proliferation of all the tissues. The cells are multiplied in 
number, the nuclei have increased, and the blood-vessels 
show the characteristic changes of specific arteritis. The 
walls of these vessels are thickened, all of the coats partici- 
pating to an equal degree. The process very often starts 
in the intima, but not invariably so, as was maintained some 
years ago by Heubner. The pia is generally adherent to 




Fig. 71. — Section through a Portion of Dorsal Cord (Ventral Surface). Marked 
thickening of pia ; cellular infiltration of same, seen best in that portion which pro- 
jects inward ; infiltration of substance of cord. 

the spinal cord, and the cellular infiltration extends from it 
into the substance of the spinal cord. This invasion is a 
very gradual one, and may begin at almost any point of the 
cross-section of the cord ; but the lateral columns are more 
frequently involved than any other region, and for this rea- 
son the spastic symptoms are, as a rule, the first to be de- 
veloped. In other cases in which the morbid process in- 
vades the posterior columns the symptoms may resemble 
those of tabes rather than those of a spastic form of paral- 



SYPHILIS OF THE SPINAL CORD. 301 

ysis. If the invasion occurs from the ventral surface, as 
it rarely does, the symptoms will naturally be of an 
atrophic order rather than of a spastic or sensory kind. 
But wherever the first point of attack may be, the morbid 
process gradually works its way inward from the periph- 
ery, and its plan of attack is generally from symmetri- 
cally situated points. The cellular proliferation and the 
slight increase in neuroglia tissue, as well as the gradual 
disappearance of the integral elements of the cord, give to 
these cross-sections, if examined under the microscope, the 
appearance of ordinary myelitis, and if it were not for the 
characteristic changes in the blood-vessels and for the evi- 
dence that the process has worked its way inward from the 
periphery, and, indeed, from the pia, the anatomical proof 
of a specific process would be very difficult to establish. 
As it is, there are not a few cases in which satisfactory 
proof cannot be given, and the pathologist is compelled to 
rely upon the clinical evidence in the case to prove the 
syphilitic nature of the disease. But the wide-spread char- 
acter of the disease, and the very gradual destruction of the 
different systems* of the cord, as well as the important part 
played by the pia and the blood-vessels, leave little doubt 
as regards the true nature of the inflammatory process, and 
at the same time help us to understand the peculiar behavior 
of the clinical symptoms. The Figures 69-71 will give full 
details of the specific meningo-myelitis as seen in one of my 
adult cases. 

The meningo-myelitis may be associated with special 
gummatous deposits in any part of the cord or in the brain. 
Thus, in one of my cases a gumma in the pons was asso- 
ciated with a wide-spread specific meningo-myelitis at the 
base of the brain and throughout the entire extent of the 
cord. If such a complication exists the child may present 
the symptoms of tumor of the brain or cord, together with 
the symptoms of extensive meningo-myelitis. 

The fact that spinal syphilis has a distinct tendency to 
improvement makes the prognosis favorable, although this 
tendency implies also a danger of relapses. But syphilis in 

* Nonne has shown that Erb's type may be due to a combined systemic degenera- 
tion involving the pyramidal, the cerebellar tracts, and the tracts of Goll. 



302 THE NERVOUS DISEASES OF CHILDREN. 

the spinal cord of the adult, as well as of the child, is amen- 
able to treatment, and the prognosis is distinctly more fa- 
vorable than in cases in which the same symptoms might 
be present and not due to syphilis. The prognosis is, for 
instance, very much more favorable than in cases of con- 
genital diplegias or paraplegias. The possibility of recovery 
should therefore be kept in mind, but the hope of complete 
restoration can be entertained only if the symptoms recede 
promptly upon anti-syphilitic treatment. If the symptoms 
do not in any way yield to treatment, and if, after a number 
of careful trials the condition remains practically the same, 
the prognosis is as unfavorable as it would be in any other 
case of myelitis. The prognosis will also depend very 
largely upon the evidence of the amount of damage that 
has been done by the specific process. If the symptoms 
show that there has been a complete destruction in one or 
more systems of the spinal cord the possibilities of recovery 
are naturally less than in cases in which the symptoms point 
to but a slight involvement of these parts. Yet whatever 
the outlook may be at the time the patient is examined, it is 
the physician's duty to warn the parents against the possi- 
bility of relapses and to prepare the relatives for the fact 
that later attacks may involve more vital parts, and may, 
therefore, be more dangerous than the one through which 
the child is passing at the time. 

Treatment. — Syphilis of the spinal cord in the child, as 
well as in the adult, calls for very prompt treatment. We 
must depend upon the usual remedies — the mercurials and 
the iodides. It is wrong to depend upon either one alone, 
for in some cases the mercurials are more effective than the 
iodides, and in others the reverse is true. It is my practice 
invariably to begin treatment with one drug,* and to stop 
mercury or the iodide if either is not well tolerated or if 
the improvement is such that I find it unnecessary to push 
either drug to the extreme. In some instances it is evident 
that all forms of specific treatment are of no avail. Mercury 
should be given in the form of inunctions, either of the ten 
per cent, oleate of mercury or of the unguentum hydrargyri. 

* I agree with Lewin that it is best not to give mercurials and iodides in combina- 
tion. 



SYPHILIS OF THE SPINAL CORD. 303 

According to the age of the child, one-half to one gramme 
of the ointment may be rubbed in daily. If iodides are to be 
exhibited begin giving small doses of the saturated solution 
of the iodide of sodium, the daily dose to be increased 
slowly. Thus I begin with three or four minims of this 
solution, to be given in milk three times a day, and increase 
the daily dose by one minim until a child, according to its 
age, takes ten, fifteen, or twenty minims of the iodide three 
times a day. As soon as decided improvement takes place 
the quantity of the mercurial inunction given may be re- 
duced and soon stopped altogether ; but the iodides may be 
pushed for some time after this improvement has been 
noticed. In cases in which no improvement occurs, in spite 
of the proper administration of these drugs,* it is well to 
stop both, for a time at least; but I would advise, under all 
circumstances, if the diagnosis is safely established, to make 
repeated trials of these drugs in the manner indicated 
above ; and if a child has passed through several attacks of 
specific myelitis, it would be well to subject it to periodic 
treatment by the iodides in the same manner that one 
would administer treatment for constitutional syphilis. In 
addition to the anti-syphilitic treatment, the physician will 
have to prescribe tonics, sufficient exercise, and nutritious 
diet, according to the needs of the patient. Moreover, in 
each attack, or as long as the paralysis lasts, it will be neces- 
sary to use electricity and massage, as one prescribes them 
in all other cases of myelitis, whatever the origin of the my- 
elitis may have been. 

* Oppenheim advocates the use of iodipin. 



CHAPTER XVII. 

DISSEMINATED SCLEROSIS. 

Disseminated, insular, or multiple cerebro-spinal scle- 
rosis is distinctly a disease of early life. The first pro- 
nounced symptoms appear, generally, in the second and 
third decades of life, but a few prodromata can be traced 
back to a much earlier period.* The disease, as described 
by Charcot, is characterized by a ver}^ definite set of symp- 
toms ; but variations from this type are not infrequent. 

Symptoms. — The prodromal symptoms of this disease 
consist of a weakness of the upper, and sometimes of the 
lower, extremities, of a slight awkwardness and trembling 
of the fingers, and of slight subjective sensory disturbances 
in the arms and legs. The chief symptoms develop gradu- 
ally, and are present from the early periods of the disease to 
the very end, but vary much in intensity at different periods 
of the disease. The most characteristic symptom is the 
tremor which is observed on voluntary motion of the af- 
fected parts. It is not observed when the parts are at rest, 
and only when a voluntary effort is made ; it is spoken of as 
" intention tremor." The tremor consists of about six to 
seven oscillations per second (Grasset). It becomes most 

* Totzke stated that some of the symptoms were manifested in two cases at birth ; 
in one case at the age of five months, in one at fourteen months, and in two at the age 
of two years ; and of thirty-one cases which the same author analyzed, a very fair pro- 
portion began between the fourth and fourteenth years. There is some reason to doubt 
whether all these cases were typical cases of disseminated sclerosis. In my own prac- 
tice the youngest child with distinct symptoms of multiple sclerosis was barely eight 
years of age at the time of observation, and her symptoms did not come on before the 
tenth year. Marie, who reported three cases of " sclerose en plaques," in infants, in 
18S3, now states that he would not diagnosticate the cases as such, and believes the 
" disease very rare in children." MQller, the most recent author (1904), claims that 
there is no post-mortem evidence to prove the existence of an infantile multiple scle- 
rosis. 



DISSEMINATED SCLEROSIS. 305 

distinct if the patient is asked to take hold of any object, 
to pass a glass of water to the lips, or to take hold of a 
pencil, as in drawing or writing. At first this tremor is so 
slight that it does not interfere with the execution of the 
simpler movements, but as it increases in intensity it be- 
comes a bar to the use of the hands and legs, and may be 
an annoyance in walking. In attempting to put a glass of 
water to the lips the water is spilt (" the patient's face and 
garments are inundated " — Marie), and in writing the 
tremor is so marked that the pen tears the paper and the 
writing becomes altogether indistinct. 

Next in importance to this tremor is the difficulty in 
speech. The patient speaks deliberately, slowly, pronounc- 
ing each syllable with unusual care (scanning speech), and 
yet a distinct tremulousness of the voice is apparent. The 
consonants 1, p, g, and r, are most difficult to pronounce. 
The words in which these letters occur become unintelligi- 
ble at a very early period of the disease. 

Ocular symptoms are very common ; we may have, at 
times, an inequality of the pupils. (Uhthoff noted this in 
eleven per cent, of his cases.) The reactions to light and 
during accommodation are diminished, and a myosis is gen- 
erally present. Nystagmus appears at a very early day, 
particularly if lateral movement of the eyes is attempted. 
Upward or downward motion is unaccompanied by this 
phenomenon. Visual disturbances are not as common as 
in tabes, but do occur frequently, according to the excel- 
lent studies made of this special symptom by Uhthoff some 
years ago. Clinically we find a narrowing of the field of 
vision, particularly of color, and a diminution of the actual 
visual power. The trouble may be unilateral or bilateral. 
These symptoms are associated with atrophic discoloration 
of the papillae, which persists even though an improvement 
in the subjective symptoms may take place. The eye symp- 
toms are so similar to those observed in hysteria, that on 
this account alone the two diseases have frequently been 
mistaken for one another. 

A distinct tremor of the tongue and a peculiar vague 
expression of the countenance are the other head symptoms 
present in this disease. Charcot described a special facies 



306 THE NERVOUS DISEASES OF CHILDREN. 

of multiple sclerosis, and refers to a vague and uncertain 
look, the lips hanging down and half open. On the whole 
there is an expression of mild stupor on the face ; this ex- 
pression is not altogether unnatural, for the mental condi- 
tion is in many cases abnormal. The memory is weak ; 
there is lack of attention on the part of the patient, who 
passes easily from laughing to crying, and vice versa. Many 
of the patients are emotional to an extreme degree, and this 
is particularly true, according to my own experience, of 
those in whom the disease begins very early in life. 

The paralytic symptoms are of a spastic order. The 
legs and arms are moved awkwardly at first, later with dis- 
tinct evidence of rigidity. A spastic or spastic-paretic gait 
is present, and is associated with those other symptoms 
characteristic of all spastic paralysis and of involvement of 
the lateral columns of the cord, viz., rigidity of the muscles 
and increase of the deep reflexes. All these symptoms 
may for a long time be slight, but as the disease progresses 
they become more and more intense ; the spasticity and 
weakness grow worse until the patient becomes completely 
bed-ridden ; and the tremor increases in intensity and inter- 
feres with the use of the extremities. Speech becomes 
almost unintelligible, the memory grows weaker and 
weaker, until a truly pitiable condition is reached. Ob- 
jective sensory disturbances are not uncommon ; Freund 
has observed them in twenty-nine out of thirty-three cases. 
In fourteen there was a mere transitory anaesthesia, while 
in six cases he states that there was a more or less perma- 
nent and complete loss of sensation. He also refers to the 
occurrence of hyperesthesia, and even to the dissociation 
of sensation, but this latter form of sensory disturbances is 
surely a great rarity. Fortunately, or unfortunately for 
many, the disease covers a long period of years, during 
which a tolerable condition of health is maintained. In 
other cases, again, the symptoms described are developed 
more rapidly, and a few others are added which are not in- 
variably present. This is true of the bulbar symptoms 
leading to difficulties not only of speech but of deglutition, 
and occasionally to interference with the respiratory func- 
tions. 



DISS EM IN A TED SCIER OSIS. 



307 



Paralysis of the ocular muscles, above all of those sup- 
plied by the sixth and third nerves, are not infrequent. Ves- 
ical trouble has been recorded by Erb and Oppenheim. 
If we add to the above symptoms the complete paralysis 
resulting from apoplectiform attacks it will be evident that 

the disease may become almost pro- 
tean in its manifestations. Para- 
doxical contractions and impulsive 
laughter are frequent symptoms. 

As important as the positive 
symptoms hitherto recorded are 
those of a negative order. Atrophy 
of the muscles, paralysis of the blad- 
der and rectum, and changes in elec- 
trical reaction are entirely wanting. 
Why such a variety of symp- 
toms occurs in this disease will 
be understood easily enough when 
we refer to the morbid anatomy. 
The course of multiple cerebro- 
spinal sclerosis is slow, and though 
invariably leading to a fatal issue, 
death is, in the majority of cases, 
the result of some intercurrent dis- 
ease. 

Etiology. — Disseminated scle- 
rosis comes on most frequently af- 
ter acute infectious diseases, after 
powerful emotions, and occasionally 
after severe injuries. Oppenheim 
has shown that the disease appears 
after intoxication by metallic poi- 
sons, while Marie believes it to be 
invariably of infectious origin, com- 
ing on after typhoid fever, pneumonia, measles, scarlatina, 
and small-pox. 

Heredity is supposed to play an important role in the eti- 
ology of insular sclerosis. In the broader sense it is true ; 
for multiple sclerosis may indeed affect children who are 
descended from a neurotic stock ; but it is extremely rare 




Fig. 72. — Degeneration of the 
Cauda Equina in Multiple 
Sclerosis. (After Taylor. ) 



3 o8 



THE NERVOUS DISEASES OF CHILDREN. 



to find the disease in both parent and child. The cases of 
Pelizaeus, quoted approvingly by some authors, are not to 
my mind typical cases of multiple sclerosis ; they belong 
rather to the hereditary form of spastic paralysis. Krafft 
Ebing, and others mention refrigeration as a frequent etio- 
logical factor. 

Pathological Anatomy. — We can infer from the name 
that the chief lesions in this disease are irregular sclerotic 
patches distributed throughout the greater part of the cen- 
tral nervous system. These patches sometimes occur in the 
brain as well as in the spinal cord ; but there is no rule de- 
termining their first appearance either in one or the other, 
and there is, therefore, as little reason for establishing a 
spinal form or a cerebral form on anatomical as there is on 
clinical grounds. The plaques have, however, a few fa- 




Fig. 73. — Sections through the Pons, Medulla, and Spinal Cord, showing Sclerotic 

Patches. (Taylor. ) 

vorite sites ; the white matter of the brain, the pons and 
medulla, the lateral columns and particularly the posterior 
columns (Ziegler, Miiller) in the dorsal and lumber seg- 
ments; but they also appear in the cortex, in the cervical 
region, and even in the cauda equina. (Fig. 73.) Charcot 
was of the opinion that the foci of disease were very rare 
in the cortex of the hemispheres or of the cerebellum; 
Taylor claims they may occur in these parts as well as 
in others. The sclerotic changes may also affect the spinal 



DISSEMINATED SCLEROSIS. 



309 



^ 



-L) >& 







roots and various cranial nerves. The optic chiasm is fre- 
quently diseased. Whether the sclerosis appears in the cen- 
tral nervous system itself, or in the nerves emerging from 
it, the character of the changes is very much the same, the 
main point being that a few, and not necessarily the major- 
ity, of the nerve-fibres are destroyed. 

These patches present a bluish-gray appearance and are sometimes 
slightly elevated, at other times the parts in which they occur appear 
shrunken and contracted. In size they vary from one twenty-fifth to one 

inch. They are, as a rule, 
a little harder than the sur- 
rounding tissue, and Morris 
calls especial attention to 
the nicety with which they 
can generally be easily dif- 
ferentiated from it by the 
naked eye. 

On microscopical ex- 
amination these patches are 
found to consist of a dense 
fibrous tissue, due undoubt- 
edly to the enormous in- 
crease of the neuroglia fi- 
bres (according to Weigert). 
Weigert insists that the pro- 
liferation of neuroglia tissue 
is more marked in this dis- 
ease than in any other. In 
these sclerotic portions the 
myelin sheaths, according to 
the stage of the disease, are 
more or less diseased, but 
the axis-cylinders in the majority of the cases remain normal. The entire 
process may, therefore, be characterized as an interstitial inflammation, and 
it is owing to the preservation of the axis-cylinder that secondary degenera- 
tion in multiple sclerosis is a rare occurrence, indeed ; although Buss has- 
recorded a descending degeneration in the lumbar cord and an ascending de- 
generation of the columns of Goll, and of the direct cerebellar tract from the 
eighth cervical segment into the medulla oblongata, but his observations are 
surely exceptional. The absence of secondary degeneration also accounts 
for the frequent absence of contractures.* 






I 






:-!'' : . : "- '/</'• ;>-'■" 




FlG. 74. — Vicinity of Locus Coeruleus. 
blood-vessels. (Taylor.) 



c, cells ; v, 



* The tremor of multiple sclerosis has been attributed by some to defective isola- 
tion (loss of sheath) of the axis cylinders, on the supposition that the nerve current 
resembles an electric current, and the axis-cylinder an electric wire-; but this is rather 
fanciful pathology. 



3IO THE NERVOUS DISEASES OF CHILDREN. 

Considering the importance which has been attached to the acute infec- 
tious diseases as an etiological factor in multiple sclerosis, it would be nat- 
ural to expect a very early involvement of the blood-vessels in this disease. 
Such an involvement has been found by Ribbert, Greiff, Cramer, and Marie. 
Taylor, a more recent author on this subject, whom I am willing to follow in 
this matter, because the autopsy in his case was on a young subject, proves 
that in the earlier stages of the disease there is a distinct increase of the smaller 
blood-vessels and capillaries. (Fig. 74.) The specimens examined also ex- 
hibited small hemorrhages and migrating white blood-corpuscles. In some 
of the vessels there was a slight thickening of the walls and an increase in 
the number of the nuclei. The perivascular spaces were dilated. The same 
changes were seen in a second case of Taylor's. In a third case there was 
absolutely no change in the blood-vessels, but in the second and third cases 
the disease had lasted longer, and it is, therefore, doubtful whether similar 
changes in the blood-vessels might not have been present also in these cases 
in the earlier period of the disease. Taylor thinks that we are not justified 
in connecting these vascular changes with the sclerotic process, for the patches 
are not distinctly related to the diseased vessels. Furthermore, vessels are 
often entirely normal in degenerated areas, and in some cases the disease of 
the blood-vessels is wholly wanting, however wide-spread the lesions may be. 

Striimpell and Miiller regard the disease as an endogenous (congenital) 
affection (say, of the neuroglia), stirred into being by various exciting causes. 

Atypical Forms and Differential Diagnosis. — It 
would be an easy matter to enumerate a large number of 
atypical forms of multiple sclerosis, for the clinical symp- 
toms as developed by Charcot are not distinct in many cases 
which have, however, a direct relation to this disease, repre- 
senting either early forms of the same, or a peculiar local- 
ization of the sclerotic patches. One variety of multiple 
sclerosis with bulbar symptoms bears a close resemblance 
to amyotrophic lateral sclerosis. But in children the com- 
bination of symptoms giving rise to such difficulty in diag- 
nosis is very rare. Then there is the bulbar, or, as Spitzka 
prefers to call it, " the oblongata type " of multiple sclerosis, 
in which the usual symptoms are not nearly as well devel- 
oped as are those pointing to an involvement of the lowest 
cerebral centres. Difficulties in deglutition, in mastication, 
and in articulation as well as phonation, are most promi- 
nent, and for a long time may obscure the usual symptoms 
of the disease. 

A hemiplegic form of multiple sclerosis has also been 
observed in the adult, but I have not yet met with it in 



DISSEMINATED SCLEROSIS. 3II 

children ; nor have I seen an apoplectiform beginning of 
the disease in children, as sometimes happens later in 
life. 

Multiple sclerosis, and forms closely allied to it in children, should not be 
confounded with transverse myelitis. Acute and chronic forms of myelitis in 
children are not rare. The acute symptoms and the inflammatory condition 
upon which they depend disappear promptly enough, but a degeneration is 
set up which leads to the development of spastic and paralytic symptoms. 
These might be taken to be symptoms of multiple cerebro-spinal sclerosis 
unless the history of the patient is carefully considered. The very acute 
onset and the involvement of the bladder, the persistence of sensory symp- 
toms for a shorter or a longer period of time, will help to differentiate myelitis 
and its secondary degeneration from insular sclerosis. 

From congenital spastic paraplegia the disease can be differentiated by a 
consideration of the mode of onset, and by the very early appearance of the 
congenital disease, for insular sclerosis does not generally come on until very 
nearly the first decade of life is passed. Moreover, in the congenital cases of 
spastic paraplegia there is frequently some cerebral defect ; and the cardinal 
symptoms of sclerosis are wanting in these cases. Westphal described a 
pseudo-sclerosis, a condition closely simulating that of multiple sclerosis ; but 
it is not an important disease, for to my knowledge it has not been clearly 
established in any other instance excepting the one reported by Westphal. 

Cases of hereditary tremor might be mistaken for multiple sclerosis, and I 
have myself seen one patient, a girl aged twelve, in whom there was much doubt 
as to whether or not this tremor, which became very much aggravated on 
voluntary motion, was the first symptom of a multiple sclerosis ; but having 
observed the patient for fully five years without the addition of any other 
symptoms referable to a multiple sclerosis, I am confident that the girl is suf- 
fering from an hereditary form of tremor, and have since learned that her 
father and an uncle have suffered from the same disease since very early 
childhood. 

The question also arises in some cases whether an insular sclerosis or an 
hysteria is present, but a consideration of the symptoms will soon remove all 
doubts as to the diagnosis. Nystagmus, scanning speech, and intention 
tremor are exceedingly rare in hysteria, while the sensory disturbances of hys- 
teria are too typical to be mistaken for the slight subjective and objective dis- 
turbances of sensation that occur in multiple sclerosis. 

In the adult the chief point in differential diagnosis is 
considered to be that between multiple sclerosis and paraly- 
sis agitans. To this we need pay comparatively little atten- 
tion, since paralysis agitans is distinctly a disease of later 
years, and is rare indeed before the age of forty; but I can- 
not forbear referring to the case of a patient, whom I have 



312 



THE NERVOUS DISEASES OF CHILDREN. 



observed many years, who, at the age of fifteen years, pre- 
sented all the symptoms of genuine paralysis agitans. As 
will be seen from the accompanying cut, the position of the 
body, the expression of the face, the position of the hands, 
are typical of paralysis agitans, 
yet the symptoms have changed 
in such a way that at the pres- 
ent time they point to a mul- 
tiple sclerosis rather than to a 
shaking palsy. (Fig. 75.) 

The patient fell at the age of five 
years, and had an attack of convulsions 
with loss of consciousness, which was 
followed by a slight weakness of the 
legs, but from this condition he rallied 
rapidly enough, and was entirely well, 
exhibiting good physical and mental de- 
velopment. He was bright in school, 
and perfectly well until the age of fifteen, 
when he met with another accident ; he 
fell from a carriage and was badly fright- 
ened. 

The first symptoms he noticed were 
pains around the left ankle, then a shak- 
ing of the left leg. Soon thereafter the 
arm began to shake. Five months later 
he noticed the same symptoms on the 
right side of the body. 

On my first examination of the boy, 
three years after the accident, I found 
him well nourished ; the muscles in good 
condition, the color of the skin normal, 
but considerable vasomotor disturb- 
ances. He exhibited the mask-like ex- 
pression of the face, and his speech, as 
well as the rhythmical tremor of the 
hands, legs, and head, were exactly those 
of a patient suffering from paralysis agi- 
tans. 

Soon another set of symptoms ap- 
peared, which proved to me that this " senile " disease, when occurring in a 
young individual, was subject to peculiar modifications, which allied it much 
more closely to multiple sclerosis, a disease common in earlier life. 

While under observation he developed characteristic nystagmus, scanning, 




Fig. 75. — Patient with Paralysis Agi- 
tans, who also exhibited some Symp- 
toms of Disseminated Sclerosis. 



DISSEMINATED SCLEROSIS. 313 

and tremulous speech. All the reflexes became greatly exaggerated. The 
typical tremor of paralysis agitans, involving the head, lips, and tongue, and 
the extremities, remained unchanged. He still exhibits the propulsive move- 
ment so characteristic of paralysis agitans. The hands have the position of 
shaking palsy, and the contractures are much like those seen in the senile form. 
Motion is almost impossible, and with the symptoms pointing in part to 
multiple sclerosis, and in part to paralysis agitans, he presents a very unusual 
appearance.* 

Prognosis. — Like many other chronic disorders of the 
central nervous system, multiple sclerosis is not a rapidly 
fatal disease. It may run a continuous course for years with- 
out seriously endangering life, but any intercurrent disease is 
likely to lead to a fatal termination. At all events, everyone 
will acknowledge that it is rare to see cases of multiple scle- 
rosis that have exceeded the age of forty-five or fifty years, 
and as for those beginning very early, in life, the facts are 
altogether too few to warrant us in stating that such pa. 
tients live much beyond the middle period of life. The 
danger to life is greatest in those cases in which the ob- 
longata type predominates. The invasion by disease of the 
vital centres might lead to early death. The invasion is, 
however, a very gradual one, and sudden death need not be 
looked for. In those cases in which such a termination is 
threatened, periods of asphyxia, of aphonia, and other 
symptoms of vagus disturbance would occur, and would 
give warning of the terminal possibilities. 

The disease is absolutely incurable, although remissions 
occur in which all the symptoms excepting the tremor or 
the nystagmus disappear, and in some cases the disease may 
come to a complete standstill for a period of several years. 
In this respect multiple sclerosis is not unlike tabes. My 
notes of the cases beginning in early life would go to show 
that the disease is even more slowly progressive than it is in 
the most favorable adult cases. 

Treatment. — Keeping in mind what was said relative 
to the prognosis of the disease little can be expected from 
our attempts at treatment. But if we cannot cure the dis- 
ease we can at least secure greater comfort for the patient. 
As the tremor is most marked when the patient is ac- 

* Schultze has reported a similar case in Virchow's Archiv, Bd. LXVIII. 



314 THE NERVOUS DISEASES OF CHILDREN. 

tive — and the disease is exhausting- at best — it is wise to 
keep the patient in bed for a prolonged period of time. The 
rest cure I have found as efficient in these chronic organic 
diseases of the central nervous system as I have in the treat- 
ment of purely functional troubles. It is often surprising 
to note how much benefit, and, above all, how much comfort, 
patients derive from a complete rest when they have been 
attempting to go about from place to place, often from phy- 
sician to physician, with their weak and shaking limbs. 

With this rest treatment I am in the habit of com- 
bining mild hydro -therapeutic procedures. Generally a 
tepid bath in the morning, followed by lukewarm and 
successively colder douches down the spine, or by the 
use of the drip-sheet. At night it is well to give a tepid 
bath without douches, as the former contributes much 
to the sleep and the general contentment of the patient. 
The galvanic current may be employed to advantage. 
Stabile currents of 10 to 15 milliamperes may be passed 
down the spine and through the extremities. The faradic 
current should, however, be avoided, as it tends to increase 
the spastic rigidity of the muscles. Massage is also in or- 
der in these cases, if intelligently adimnistered. Spastic 
rigidities yield a little to such manipulations, and accord- 
ing to careful investigations of Mitchell and his son, the 
general condition of the patient is improved by massage. 
If treatment is directed in this way and adapted to each 
individual case it will be far more satisfactory than send- 
ing the patient away to various springs. The Frenkel 
method of exercises, that has proved itself to be of great 
value in tabes, is certain to be helpful in these cases, if in- 
telligently applied. 

Various drugs have been employed and recommended in 
cases of multiple sclerosis. Among these nitrate of silver, 
the iodides, and mercurials are the most prominent. I am 
confident that the first never does any good excepting pos- 
sibly to allay pain, and the last two will not be needed, for 
it is very certain that multiple sclerosis is rarely developed 
on a syphilitic basis, and there is no indication for the use 
of resorbents. 



CHAPTER XVIII. 

COMPRESSION OF THE SPINAL CORD— POTT'S PARALYSIS. 

Injury to the cord may be due to specific diseases in 
the bone, to compression by an aneurism or to growths orig- 
inating in the membranes. These conditions are very rare, 
however, in children, and whenever the symptoms point to 
compression of the cord, caries of the bone may be taken to 
be the primary trouble. Pott's disease of the spinal column 
begins so insidiously that the recognition of it and of the 
symptoms resulting from compression are reached frequent- 
ly by exclusion only. While the disease may develop at 
any period, it is more common in childhood and in early 
life; it is the result of a tubercular or scrofulous diathesis; 
and evidence is easily adduced in the majority of cases of 
the existence of such diathesis in the same family and in the 
same or in preceding generations. The slightest traumat- 
ism may be sufficient to bring about marked disease of the 
bone, but evidently only in those children who are predis- 
posed to such disease, so that here as elswhere the trau- 
matic incident simply helps to bring out the latent tendency 
to disease. There is often a period of weeks or months 
between the time of injury and the first symptoms of the 
disease. In consequence of the caries of the bone, the bodies 
of the vertebras become softened and are displaced, two or 
three of them, or even more, forming a marked angular 
curvature. Such angular curvature may in rare cases be 
the result of a septic process, in which case an abscess may 
have formed in the bone and caused the softening. Gen- 
erally the bodies of the vertebrae become diseased and the 
intervertebral substance undergoes inflammatory changes 
as well. Vertebral disease may lead to retropharyngeal, 
mediastinal and psoas abscesses. 



316 THE NERVOUS DISEASES OF CHILDREN. 

The deformity resulting from the disease of the bone is 
of such a character that a sharp angle is usually formed at 
one point, at which the cord is generally much compressed. 
In some instances the cord becomes quite thread-like. It is 
remarkable that the cord may sustain a very large amount 
of compression before its functions are seriously interfered 
with, or the symptoms of disturbed function appear. This 
accounts for the fact that in many instances the angular 
curvature may be extreme and yet neither paralysis nor 
anassthesia is observed during life. Lateral displacement 
may occur without its having any influence upon the cord. 
Very naturally the spinal cord suffers under these condi- 
tions, and the roots emerging or entering through the in- 
tervertebral foramina are compressed by the thickened 
sheaths or by other inflammatory products. This accounts 
for the frequent occurrence of pain in these cases, and for the 
occurrence of paralysis in cases in which the cord itself 
shows tolerably normal structure. 

The causes of injury to the spinal cord in the subjects of 
Pott's disease are twofold : first, the cord is actually com- 
pressed in many cases, and, secondly, it suffers from the in- 
flammatory products gathering between the cord and the 
bony parts. The latter cause is often quite as active as the 
former. The changes in the cord itself will vary very 
largely according to the degree of compression. The cord 
is somewhat altered, the normal white having given way to 
a grayish or reddish-gray tint. The cord is less hard than 
under normal conditions, both these changes being clearly 
due to inflammatory processes. It has been found by 
Schmauss and others that the amount of inflammation of 
the cord is not always in keeping with the degree of com- 
pression. Recent French authors have attached consider- 
able importance to an oedema of the cord produced by 
compression of the veins as they issue from the cord. The 
oedema is subject to fluctuations ; and a diminution of the 
same may be responsible for an improvement in the pa- 
ralysis. 

The symptoms of Pott's paralysis often set in long be- 
fore there is any possibility of actual compression. In those 
cases we must suppose that the symptoms are due to a 



COMPRESSIO.V OF THE SPIXAL CORD. 317 

pachymeningitis constricting the spinal roots and blood- 
vessels ; the occurrence of rapidly developing spinal abscess, 
associated with painful paralysis, and the disappearance of 
the paralysis as soon as the abscess is emptied, lend support 
to this view. By degrees an extension of the inflammatory 
process takes place into the substance of the cord. The 
myelitis as it occurs with Pott's disease shows a general in- 
crease in the interstitial tissue, with proliferation of the 
various cells and degenerative changes in the more impor- 
tant nerve elements. Granular corpuscles, corpora amylacea, 
and globular masses of myelin are found in sections that are 
properly hardened. The gray matter may have become 
almost indistinguishable from the white, and the larger 
ganglion cells may have entirely disappeared from the for- 
mer. In addition to these changes the usual ascending and 
descending degenerations will occur, as from any other 
focus of inflammation in the cord. The changes in the pe- 
ripheral nerve-roots will be those that would be expected 
from actual compression and inflammation. The interstitial 
tissue will be increased, the nerve-fibres themselves will be 
wasted, and the axis-cylinders will show the usual changes 
of degeneration. 

Symptoms. — The chief symptoms in this disease are a 
very gradual development of a spastic paralysis, generally 
of the legs and possibly of arms and legs ; the reflexes are 
increased, and radiating pains are present at a very early 
period of the disease. The symptoms often resemble those 
of a dorsal myelitis, since Pott's disease attacks the dorsal 
region more frequently than any other. If other regions 
of the spine are diseased the symptoms will vary as the}' 
would in the various forms of myelitis, but root symptoms 
will always play a very prominent part. The symptoms 
due to compression of the root - fibres will also help to 
differentiate between a myelitis following Pott's disease 
and the ordinary traumatic or non-traumatic myelitis. The 
compression of the sensory nerve-roots causes severe pains. 
It may also interfere with normal sensation. (Dissociated 
sensation has been noticed in several of my cases); anaes- 
thesia dolorosa may be associated with the paralysis. Be- 
fore the condition of anaesthesia is established a transitory 



318 THE NERVOUS DISEASES OF CHILDREN. 

hyperesthesia may have existed. The irritation of the 
motor root-fibres would lead us to expect a condition of 
involuntary contractions of the muscles, but, as a matter of 
fact, these are not so frequent, and probably because the 
ventral gray matter of the cord, being nearest the verte- 
bral bodies, is affected at an early day ; and since the func- 
tion of this gray matter is impaired, irritation of the an- 
terior roots connected with it will not be able to exhibit 
symptoms of irritation. According to the location of the 
disease in the bone the symptoms may for a time remain 
unilateral ; but, as a rule, the disease spreads rapidly 
enough to bring about a bilateral, and often symmetrical, 
set of symptoms within a very short period of time. Fur- 
thermore, as the disease progresses the paralyzed parts may 
become atrophied, the vesical and rectal reflexes may be 
impaired, and we then have all the ordinary symptoms 
of myelitis, even including bed-sores and the usual trophic 
disturbances of the skin. 

The course of the disease may be inferred from the ra- 
pidity with which all these symptoms are developed. If 
the disease is of a slowly progressive character, the symp- 
toms will naturally be developed one by one, whereas if the 
bone disturbance is of a more pernicious and rapidly pro- 
gressive character the symptoms of a complete myelitis 
will be reached at a very early day.* 

Diagnosis. — Paralysis due to Pott's disease must be 
distinguished from subacute or chronic myelitis. This dis- 
tinction is not always an easy one to make. It is reached 
most readily in those cases in which the course of the dis- 
ease is a very slow one and the usual causes leading to a 
transverse myelitis are wanting. It is rendered probable 
by the coexistence of pain with the paralysis, and above all 
things by the discovery of tenderness to pressure over the 
spinous processes. These same symptoms might occur in 
cases of tumor of the cord, but the unilateral development 
of symptoms in the latter disease will soon lead to a safe 
differential diagnosis. (See next chapter.) The earlier 
symptoms of a Pott's paralysis may simulate the condition 

* In some cases, like the one reported by Daxenberger, the caries may be latent for 
years, before spinal-cord symptoms are developed. 



COMPRESSION OF THE SPINAL CORD. 3I9 

of spastic paraplegia, either of the congenital order or of 
that form due to specific disease. 

In several cases, under the author's care, the symptoms of Pott's paral- 
ysis were developed long before any actual evidence of disease of the verte- 
brae could be elicited. Whitman also states that in a number of cases the 
paralysis precedes the deformity. The diagnosis must at times be reached 
by exclusion ; but the condition may be suspected in cases of a gradually de- 
veloping spastic and painful paralysis, in which there is no trace of preceding 
injury, and no history of any acute infectious disease immediately preceding 
the onset of the symptoms. 

The age of the patient and his general appearance will 
also help to establish a differential diagnosis. 

Prognosis. — The progress of the paralysis will depend 
mainly upon the development of the disease in the bone. 
If the disease has lasted long enough to produce complete 
compression at one point, the spinal-cord symptoms may per- 
sist during life, although the bone disease be checked or re- 
covered from. In other cases the course of the spinal-cord 
disease may be checked, although the disease of the bone 
steadily progresses. With the subsidence of the inflamma- 
tion the sensory symptoms may diminish, but the condition 
of paraplegia may continue years thereafter as a natural re- 
sult of the changes that have been set up in the spinal cord. 
In a number of cases, however, there is a proportionate de- 
velopment between the disease of the bone and the disease in 
the spinal cord. In the more severe cases the paralysis re- 
mains unaltered, or is steadily progressive, and all those 
complications arise, such as bed-sores, cystitis, and the like, 
which help to bring about a fatal issue in many cases of my- 
elitis. In the milder cases recovery is to be expected, and 
many a child who has not been able to walk for years during 
the active period of caries has attained a tolerably normal 
gait in later life. On the whole, the motor symptoms are 
much more persistent than the sensory. The prognosis is 
much more grave in the cases of cervical Pott's disease than 
in those occurring in the lower vertebral regions, for rea- 
sons which can be readily understood. 

Treatment. — In the treatment of paralysis resulting 
from Pott's disease surgical methods should be resorted to 



320 THE NERVOUS DISEASES OF CHILDREN. 

at the very beginning. If the sprnal cord is to recover, the 
tubercular process in the spinal column must be checked 
first of all. The old method of absolute rest should be em- 
ployed, and tonics, such as cod-liver oil and iron, should be 
given. I do not wish to enter into the discussion as to which 
of these measures, the rest or the tonics, is more impor- 
tant. Some will be cured by the one, and some by the other, 
method, but I have not the slightest doubt that if all these 
measures are employed conjointly the result will be more 
satisfactory. In addition to rest, absolute immobility should 
be secured, either by forcible extension in bed, or by the 
application of a plaster-of- Paris jacket. The plaster-of- 
Paris jacket is not so useful during the period of rest as it is 
during the period of partial recovery, in which it is desira- 
ble for many reasons that the child should be taken out of 
bed and enabled to walk in the open air. I am very certain 
that the application of counter-irritants, which is still recom- 
mended by many, is of no use, and simply helps to increase 
the tortures that these children suffer. Of late years active 
surgical interference has been suggested in these cases by 
Macewen, Horsley, and others. Thorburn and Chipault's 
statistics show a mortality of forty-three per cent. The sur- 
geon's skill should, at all events, be restricted to those cases in 
which the clinical symptoms prove that there is evidence of 
compression at any one special point by inflammatory prod- 
ucts or by displaced bone. If the child is strong enough to 
tolerate such surgical procedures, the removal of these 
products of inflammation may be attempted ; but it is well 
to caution the physician who may be eager to try new sur- 
gical procedures that children are less tolerant than adults 
of spinal and cerebral operations. Callot's redressement 
forcee cannot be endorsed. 

The orthopedic surgeon, by well planned tenotomies, may 
enable the child to use its feet to greater advantage. His 
treatment will be supported in every case by the discreet 
use of electricity and massage. The ordinary blood and 
nerve tonics will help very much more than the iodides 
or mercurials, but these should be given in the earliest 
stages of the trouble. I have seen favorable results from 
mercurials in cases in which the process was assumed to be 
gummatous and not tubercular. 



CHAPTER XIX. 

TUMORS OF THE SPINAL CORD AND ITS MENINGES. 

The study of tumors of the spinal cord was at one time 
considered to be of small practical value on account of the 
rarity of the affection ; but far greater interest attaches 
to it now from the fact that the removal of these tumors 
has been rendered possible by the recent advances in sur- 
gery. About fifteen years ago Mills and Lloyd collected 
and analyzed fifty cases of cord tumors ; fourteen per cent, 
of these were under twenty years of age. Bruns, Oppen- 
heim, Schlesinger, and others speak of the relative fre - 
quency of occurrence of these tumors in the early years of 
life. 

Causes. — The actual cause of tumors of the spinal cord can be deter- 
mined as little as in the case of cerebral tumors. They are rarely primary, 
and are commonly due to some constitutional diathesis, such as tuberculosis 
or syphilis, or are the metastatic products of malignant tumors in other organs 
of the body. The tendency to proliferation of nerve-tissue is not so marked 
in the spinal cord as in the brain of the child. Gliomata originate in the vicinity 
of the central canal, and here are of special interest, as they often break down 
and lead to the formation of cavities, and hence have given rise to the discus- 
sion whether these cavities are invariably the result of such disorganization 
of a neoplasm, or whether the condition of syringo-myelia may be the result 
of congenital enlargement of the central canal. Small hemorrhages and em- 
bolism, though much rarer than in the brain, may give rise to cystic forma- 
tions, but among cystic growths those due to parasitic infection, particularly 
to the echinococcus, are the most common. As in the case of brain tumor, 
accidental injury has been declared responsible by many for the occurrence of 
spinal tumors. It is doubtful, however, whether traumatism plays any further 
role than that either of eliciting a latent tendency to such disease, or of 
merely calling attention to the symptoms of the disease which may have 
antedated the injury. 

Symptoms. — The symptoms of tumor of the cord might 
be deduced from a consideration of the disturbance of 



322 THE NERVOUS DISEASES OF CHILDREN. 

function due to a very gradual invasion of the cord. Thus 
in many cases the symptoms are strictly unilateral for a 
time, and become bilateral with the extension of the disease 
from one part of the cord to the other. The symptoms will 
naturally vary also according to the seat of the disease, ac- 
cording to the more or less rapid destruction of the cord, 
and according to the development of the tumor in the 
meninges or in the spinal cord itself. It is of the greatest 
importance to distinguish between extra-medullary and me- 
dullary tumors. Extra-medullary tumors are characterized 
by the early appearance of root symptoms, more persistent 
pain in the back, and by sensory disturbances which point 
to compression of the posterior root-fibres ; by paralysis 
and atrophy if the growth is on the ventral surface. These 
distinctions will not hold good if, as in. a case of my own, 
the intra-medullary tumor starts near the entrance of the 
posterior root-fibres and actually compresses these. In 
some cases, however, the disproportion between the sen- 
sory symptoms and the paralysis will direct attention to 
the seat of the tumor outside of the spinal cord, particu- 
larly if all symptoms pointing to involvement of the cen- 
tral gray matter are absent. The focal symptoms in ex- 
tra medullary, as well as in intra-medullary tumors will 
vary greatly according to the seat of the tumor. There 
may be but slight sensory disturbances in a single area 
or in one limb; the sensory disturbances may be uni- 
lateral, and these may or may not be associated with par- 
tial paralysis, either unilateral or bilateral ; or there may be 
crossed sensory and motor symptoms. Any set of spinal, 
and more particularly hemi-spinal symptoms, whether of a 
motor, sensory, or vasomotor order, if they be slowly pro- 
gressive, are suspicious of tumor. There will be additional 
reason to suspect tumor if there is evidence of a distinct 
constitutional diathesis. All the symptoms of various 
spinal-cord affections may be simulated by tumor, accord- 
ing to the part of the spinal cord affected, and in this 
respect the symptomatology of tumors may be quite as 
varied as that of syphilis of the spinal cord. 

If we endeavor to analyze the symptoms in detail, we 
find that disturbances of sensation are by far the most com- 



TUMORS OF THE SPIXAL CORD AND ITS MENINGES. 323 

mon. If the tumor is in the cervical region, the pain radi- 
ates into the upper extremities, and generally into definite 
parts of the brachial plexus ; if in the dorsal region, the 
pain is relegated to the region of the ribs, the thorax, or the 
abdomen ; if in the lumbar region, the pain is described as 
limited to the various parts of the lumbar or sacral plexus. 
Whatever the level of the lesion may be, the sensory dis- 
turbances may be unilateral for a time and gradually be- 
come bilateral. Vasomotor disturbances occur, similar to 
those in peripheral neuritis ; the skin becomes glossy, livid, 
and painful. With these symptoms muscular wasting and 
paralysis will soon be associated. As the disease progresses 
the hyperalgesia or the paresthesia gives way to complete 
loss of sensation, the paralysis becomes complete, and the 
vasomotor disturbances increase, and serious trophic dis- 
turbances may set in. The sensory changes may vary in 
still another respect. Since the tumor need not invade all 
sensorv fibres at once, some may remain entirely intact, 
while the function of others becomes seriously impaired. 
Thus dissociated sensation may be present in some cases, 
but is rarely as distinctly developed as in the case of syringo- 
mvelia, which, as we have seen above, may or may not be 
connected with the formation of a central neoplasm. Ac- 
cording to well-known physiological laws, and to the old- 
time teachings of Brown-Sequard, one would expect in 
many of these cases of tumor to find distinct symptoms of 
unilateral lesion of the cord, namely, paralysis in one mem- 
ber or in one-half of the body, with loss of sensation in the 
opposite half ; but this, curiously enough, is not often the 
case. 

The paralysis will be developed in accordance with the 
ordinary laws relating to spinal-cord affections. The mus- 
cles represented in the segment or segments involved by 
the neoplasm (see tables in Chapter I.) will not only be 
paralyzed, but will also undergo atrophy, while the mus- 
cles represented in lower segments will show spastic forms 
of paralysis without any atrophy. The changes in electri- 
cal reactions will also vary in accordance with these con- 
ditions, the onlv muscles exhibiting distinct reaction of 
degeneration being those governed by cells in the affected 



324 THE NERVOUS DISEASES OF CHILDREN. 

segments. Spasmodic contractions, the ordinary rigidities 
and contractures, will be observed in these cases under the 
same conditions as in cases of myelitis. All the symptoms 
will be largely influenced by the fact that an active my- 
elitic process is started up sooner or later in the vicinity of 
most of the tumors. This is particularly true of tubercular 
and gummatous deposits, whereas other forms of tumor may 
exist for some period of time without seriously affecting the 
tissue in their vicinity. 

Paralysis of the sphincters of the bladder and rectum 
will occur in cases of tumor as in cases of myelitis. In the 
case of tumor in the lumbar region, absolute paralysis of 
the sphincters and retention are the natural results, while 
if the tumor is above this level incontinence will be the 
more frequent condition ; but in the later stages retention 
with overflow is the rule. The conditions of the sphincter 
ani will vary in very much the same way. Tumors of the 
cauda equina deserve especial consideration. In addition 
to the general symptoms, pains radiating into the sacrum, 
the perineum, the anus, the bladder, and into the distribution 
of one or both sciatic nerves will be present. In one of my 
own cases, bladder and rectal symptoms were absent for a 
long time. The gait may be only slightly defective for 
some weeks ; with the growth of the neoplasm the paralysis 
of the legs will become more and more evident. In two 
patients whose cases were reported by the author, pressure 
to one side of the median line had produced the precise 
sensory disturbances of which they had complained. 

It is well to remember that a very large number of so-called " spinal cord 
tumors " start from the vertebral bodies ; many others from the meninges, 
and that the spinal symptoms are in reality compression-symptoms, and not 
due to an invasion of the cord. Not infrequently in children, as I had occasion 
recently to see in a lad of fourteen years, the spinal tumors are so clearly 
metastatic products (sarcomata) that operative interference would be of no 
avail. In some instances of tuberculosis, spinal symptoms may appear to be 
primary; but the autopsy will reveal the more probable focus of infection. In 
a case of solitary tubercle of the cord between the sixth and seventh cervical 
segments I found in addition that the cord between the seventh cervical and 
the fourth dorsal segments was a diffluent mass. There were also minute 
tubercular deposits discovered in both lungs, and in both apices there were 
incipient cavernous spaces. 



TUMORS OF THE SPINAL CORD AND ITS MENINGES. $2$ 

Pathology. — The morbid anatomy of tumors of the 
cord differs very little from that of tumors of the brain. 
The central nervous system is in this respect a unit, and the 
same growths which are apt to occur in the brain occur, 
though with lesser frequency, in the spinal cord. In the 
cases of Mills and Lloyd, only four were of a tubercular 
character, while twelve were either sarcomata or gliomata. 
Parasitic growths occurred in three cases of the fifty, syph- 
ilitic growths in five, and the remainder were distributed 
among various kinds of neoplasms, such as myxoma, fibroma, 
carcinoma, and psammoma. Neuromata of the cauda equina 
must be included in the list. The largest collection of 
cases of solitary tubercle of the spinal cord has been made 
and carefully analyzed by Dr. Herter. His experience 
proves that the disease is one of early life, twenty of the 
twenty-six cases occurring before the age of thirty-five. 
A few authors have reported cases of tubercle w T hich they 
supposed to be primary ; but, as in the case reported by 
myself, although it was difficult to prove any other focus of 
tubercular disease during life, such focus of disease evi- 
dently precedes the spinal disease in every instance. Spe- 
cific infiltration of the spinal cord is, on the whole, more fre- 
quent than the same infiltration of the brain. To be sure, 
relatively few cases of this description are on record, but I 
am certain that more careful examination will hereafter re- 
veal a larger number of gummatous deposits, if not gum- 
matous growths, in different parts of the cord, associated 
with the specific meningo-myelitis, which is, as has been 
stated in another chapter, the most frequent form of syphi- 
litic disease of the cord. Tumors of the cord may occa- 
sionally erode the surrounding bony parts and may work 
their way to the surface, but this is very much rarer indeed 
than a similar invasion of the skull in cases of brain tumor. 
In a case of my own the tumor (though a small one) " pre- 
sented " though an intravertebral foramen. 

Diagnosis. — Tumors of the spinal cord are to be differentiated from 
hemorrhage into the cord, from spinal caries with Pott's paralysis, from 
pachymeningitis cervicalis, transverse myelitis, syringo-myelia, neuritis, and 
from injuries of the cord. 

In the case of hemorrhage the paralysis and other symptoms resulting 



326 THE NERVOUS DISEASES OF CHILDREN. 

therefrom appear with great suddeness. The condition is one of extreme 
rarity, and in many of the cases leads to complete paralysis, with all its com- 
plications, and to death in a relatively short period of time. A confusion can, 
therefore, exist only in the first few days of the disease, and under such cir- 
cumstances the suddenness of the onset would argue strongly in favor of 
hemorrhage. 

Spinal caries, with the resulting paralysis, may occasionally present symp- 
toms similar to those of spinal tumor. In both, pressure symptoms are pres- 
ent, but in the case of caries of the bone the symptoms are not nearly so 
intense, as a rule, as in those due to the presence of neoplasm. The chief 
reliance in differential diagnosis must, however, be placed upon the detection 
of bone trouble in the case of spinal caries, although, as we have seen, such 
evidence does not always precede the onset of pressure symptoms. The 
more rapid course of the symptoms in spinal tumor, and the negative results 
of treatment, will also help to differentiate the two conditions. In caries of 
the vertebras, symptoms pointing to the ventral (anterior) surface are a little 
more frequent than in tumor. 

As between pachymengitis cervicalis and tumor of the cervical region, there 
are but few points of differential diagnosis, and the diagnosis will have to 
rest very largely upon the excessive rigidity, the very typical distribution of 
the paralysis in the hand, and the more acute pain in cases of pachymenin- 
gitis than in those of tumor. In tumor, moreover, the symptoms are more 
apt to be unilateral for a time, while those of pachymeningitis are, as a rule, 
bilateral and symmetrical. 

Some of these same points of differential diagnosis will help us also to 
distinguish between transverse myelitis and tumor of the cord, the chief 
points of difference being these : In myelitis the onset is much more rapid 
than in tumor, there is less pain, and the entire cross-section of the cord 
shows symptoms of involvement very much more promptly in myelitis than 
in the majority of cases of neoplasm. A doubt as to which of these two 
conditions is present can exist in the first few days or weeks only ; after this 
the general course of the symptoms will determine the character of the morbid 
process. 

Syringo-myelia is closely related to tumor of the cord ; there is doubt, in 
fact, whether some, if not many, of the cases of syringo-myelia do not repre- 
sent primary neoplasm of the cord, with secondary enlargement of the central 
canal and a breaking down of the spinal tissues. But the gliosis which may 
or 'may not be the starting-point of a syringo-myelia, takes an extremely 
chronic course, and it is, therefore, by this fact alone, as well as by others, 
that the two diseases can be sufficiently distinguished from one another. In 
syringo-myelia the symptoms are often strictly bilateral, or symmetrical, and 
the disturbances of sensation (the loss of pain and temperature sense, while 
the tactile and muscular sense are preserved) are very typical of syringo-my- 
elia and are rarely met with in cases of spinal tumor. In syringo-myelia 
there is also evidence in many cases of a slowly ascending or descending 
morbid process and such slow ascent or descent is not common in cases of 
tumor. The differential diagnosis is one of great importance, for the one 



TUMORS OF THE SPINAL CORD AND ITS MENINGES. 32/ 

disease is compatible with prolongation of life, while the other is rapidly de- 
structive. (See end of this chapter.) 

The symptoms of neuritis may for a time obscure those due to tumor. 
In cases which I have observed the first symptoms that appeared were those 
of a neuritis involving various branches of the brachial plexus. These were 
due to compression of the spinal roots, and are naturally present in those 
cases in which the tumor begins either in the meninges or near the surface 
of the cord. The early appearance of complete paralysis and the develop- 
ment of other symptoms, particularly those pointing to interference with the 
vesical and rectal reflexes, will argue in favor of neoplasm and not of a sim- 
ple neuritis. The rapid progress of the symptoms in the case of tumor, the 
entire absence of fever in most of them, will also point to tumor rather than 
to neuritis. The presence of sensory and paralytic symptoms in both halves 
of the body, or in the lower as well as upper extremities, without involvement 
of the bladder or rectum, may be considered as pointing to a multiple neuritis 
rather than to tumor. 

A localized specific meningitis may at times simulate tumors of the cord, 
and, as was indicated before, the frequent association of gummatous de- 
posits with such specific meningo-myelitis may present unusual difficulties of 
differential diagnosis. In one case under my observation for a period of 
several months, it was impossible to differentiate accurately between these 
two conditions ; and unfortunately the result of treatment is not to be de- 
pended upon, for even in cases of a distinctly specific character the symptoms 
do not always yield to antisyphilitic treatment. 

Severe injuries of the spinal cord may present symptoms resembling those 
of tumor. But the history of the case, and the extreme painfulness over 
the spinous processes, will prevent the possibility of confusion between the 
two. 

Prognosis. — In spite of increasing- surgical successes 
tumor of the cord is an extremely grave disease. Spinal 
growths are secondary to malignant disease in other parts 
of the organism and help to intensify the gravity of the dis- 
ease elsewhere. In the case of tubercle, sarcomata, and car- 
cinomata the prognosis is absolutely bad, while it is only a 
little better in cases of gummata. 

The duration of the disease varies according to the site 
of the tumor and according to the morbid character of the 
new growth. Tumors of the cervical region are, on the 
whole, more rapidly fatal than those in the dorsal or lumbar 
region. Tubercles, carcinomata, and sarcomata, take a more 
rapid course, as a rule, than the other forms of neoplasm. 

Treatment. — Tumors of the spinal cord and its me- 
ninges cannot be sensibly affected by drugs. There is a 



328 THE NERVOUS DISEASES OF CHILDREN. 

possible exception in the case of gummata; yet, as was 
intimated above, the effect of specific treatment in these 
cases is, to say the least, extremely doubtful. I would ad- 
vise, however, that every case of tumor of the cord be 
given the benefit of the doubt, and that active specific 
treatment by iodides and mercurial inunctions be attempted. 
If these drugs avail little, no other drug need be exhibited. 

Much has been expected of surgical treatment; contrary 
to our experience with brain tumors, these expectations 
are nearer fulfilment. Extra-medullary growths are the 
only ones that we can expect the surgeon to remove. Suc- 
cess has been reported by Gowers, Horsley, Starr, Bruns, 
Oppenheim, Spiller, myself, and others.* The cases most 
favorable for operation are those which occur near the sur- 
face of the cord or in the meninges, and those in which the 
operation is done at a relatively early day, before the neo- 
plasm has invaded the cord or has been followed by sec- 
ondary myelitis and secondary degenerations. The cases 
published by Laqueur, in which the tumor was found out- 
side of the dura mater and compressing the cauda equina, 
those reported by Oppenheim, Dejerine, Fraenkel, Putnam, 
Boettiger-Krause, and myself, make it almost incumbent 
upon us to urge operation in extra-spinal tumors. 

The question arises whether extirpation of the tumor 
should be attempted in cases of a tubercular nature and 
in those in which gumma is suspected. In tubercle of the 
cord, even the successful removal would in all probability 
prolong life but very little, so that surgical interference is 
scarcely warranted ; and yet in a case that is otherwise fa- 
vorable for operation there seems to me to be sufficient rea- 
son to interfere, if there are no other symptoms of general 
tuberculosis. I am in favor of removing gummata if the 
tumor has resisted, as it generally does, every form of treat- 
ment, provided such treatment has been carried out relig- 
iously for a period of from three to four weeks without any 
sign of improvement. I consider Horsley 's advice entirely 
sound, that if a tumor has resisted every form of treatment 
for a sufficient length of time, and if the conditions are 

* Oppenheim states that in 17 of 46 tabulated cases (36 per cent.) cure or improve- 
ment was obtained as a result of surgical interference. 



TUMORS OF THE SPINAL CORD AND ITS MENINGES. 329 

favorable for surgical interference, the proper surgical pro- 
cedures should be resorted to, and that, too, without much 
delay, for delay is far more serious under these circum- 
stances than prompt and proper surgical interference would 
be. 

SYRINGOMYELIA AND GLIOSIS OF THE CORD. 

Syringomyelia, or, more properly speaking, " myelosyringosis," is a form 
of disease that is rare enough in the adult, and still rarer in children. Its 
symptoms resemble somewhat those of tumor of the cord, of amyotrophic lat- 
eral sclerosis and of progressive muscular atrophy. As the name indicates, 
the disease is due to cavity formation in the spinal cord. This may be the 
result of a congenital abnormality of structure, or it may be due to a glioma- 
tous process starting in the vicinity of the central canal, and causing a de- 
struction of tissue. (See monographs by Hoffmann and Schlesinger.) 

\Ve need not discuss whether the one or the other mode of origin is the 
more frequent, and on this point opinions still vary. The cavity is, as a rule, 
largest in the cervical segments, but may be continued up into the medulla and 
downward into the dorsal and lumbar segments. 

Symptoms. — Less than fifteen years ago syringomyelia was considered a 
mere curiosity without any special practical importance ; but the investigations 
of Schultze and of Kahler have enabled us to recognize the disease by a very 
definite set of symptoms. The symptoms will naturally vary according to the 
extent of the cavity ; but as the cavity is generally most developed, and has 
its beginning in the cervical region, the symptoms are first observed in the 
upper extremity and generally around the shoulder. 

We may divide the symptoms into three groups : The first group consists 
of trophic disturbances, affecting the skin, its subcutaneous tissues, and the 
bones. Glossiness of skin, particularly of the fingers, deep fissures, felons — 
which are sometimes painless — and phalangeal necrosis, with marked distor- 
tion of the fingers, are the most frequent. Patients also complain of burning, 
prickling feelings, and of a sensation of numbness. These various disturb- 
ances are associated at an early date with the second group of symptoms, con- 
sisting of partial disturbances of sensation. There is either a diminution or a 
complete abolition of the sense of pain and of temperature, while the sense of 
touch and of muscular innervation remains absolutely normal. 

The third group of symptoms includes a progressive atrophy of the mus- 
cles, beginning in the small muscles of the hand, and gradually involving the 
forearm, the arm, and the shoulder muscles. The resemblancce to the Aran- 
Duchenne type of progressive muscular atrophy is very great (see Chapter 
XXII), and it is only by the association of this progressive muscular atrophy 
with dissociated disturbances of sensation, and with the trophic symptoms 
described above, that we can differentiate between syringomyelia and progres- 
sive muscular atrophy, as well as amyotrophic lateral sclerosis. In progressive 
muscular atrophy of the Aran-Duchenne type, there are no marked disturb- 
ances in the skin and subcutaneous tissue ; and in amyotrophic lateral scle- 



330 



THE NERVOUS DISEASES OE CHILDREN. 



rosis there are no disturbances of sensation, and no marked trophic symptoms, 
but in this disease and in syringomyelia the reflexes may be exaggerated. 

In syringomyelia other symptoms occur, which will depend largely upon 
the extent of the cavity formation. If the cavity encroaches very largely 
upon the anterior gray matter, there will be a large amount of flaccid atrophy 
and paralysis, the electric reactions will be disturbed, and the reflexes will be 




Fig. 76.— Section through Seventh Cervical Segment, showing Gliosis of Cord. 

(Herter.)* 



diminished in the parts governed by the affected region. Fibrillary tremor is 
present in some cases. All these symptoms are generally most pronounced 
in the upper extremities, since the cavity is greatest in the cervical region, and 
if the lateral columns in the cervical region are involved, we may expect a spastic 
paralysis of the lower extremities with increased reflexes, rigidity, and con- 
tractures. Cutaneous reflexes are, at times, normal, at other times diminished 
or entirely absent. Hyperidrosis or hypidrosis (unilateral or bilateral) have 
been reported. In the cervical cases a narrowing of the palpebral fissure and 

* This and the following figure are reproductions of specimens prepared and pho- 
tographed by Dr. Herter, and kindly furnished me for use in this book. 



TUMORS OF THE SPINAL CORD AND ITS MENINGES. 33 r 

contraction or dilatation of the pupils must be added to the. possible symptoms 
of this curious disease. If the affection extends into the medulla and pons, 
disturbed sensation in the area supplied by the trigeminus is present, together 
with atrophy and paralysis of the tongue, hoarseness, difficulty of deglutition, 
and disturbances of the senses of taste and hearing. Other cranial nerve 
nuclei may be involved ; nystagmus may be present ; polyuria and increased 
salivation have also been noticed. Pains and rigidity in the upper portion of 




FlG. 77.— Section through Part of Fourth Dorsal Segment. The central canal is in- 
vaded by glia cells and surrounded by groups of cells and nuclei resembling those of 
the ependyma. (Herter.) 



the spinal column are not infrequent, and scoliosis or a combination of scoli- 
osis and kyphosis may also be present. Vesical and rectal disturbances are 
at times superadded. 

Syringomyelia is practically a disease of adolescence and of adult life, but 
some of its symptoms can at times be traced back to the second decade of 
life. The earliest case which I have seen was in a woman of twenty, in 
whom the first symptoms began at the age of fifteen. It is interesting to 
note, however, in this connection, that a disease which is intimately related 



332 THE NERVOUS DISEASES OF CHILDREN. 

(at least in its pathology) to syringomyelia, has been observed by Dr. Herter 
in a child one year of age. Through his kindness I am enabled to insert a 
summary of his (unpublished) case, which he reported to the New York Neu- 
rological Society. 

Three months before the child was examined the right arm and hand 
began to be weak, and the head drooped forward. The examination brought 
out the following points : Complete loss of power and atrophy of muscles in 
both arms and shoulders ; the muscles were flabby ; there was slight move- 
ment of hands and fingers ; rigidity of neck ; the knee-jerks were increased, 
especially on the right side ; ankle clonus was elicited on both sides ; there 
was an irregular rise of temperature, ranging between ioo° and 105 F. daily. 
The child died two weeks after examination. 

The post-mortem examination revealed considerable enlargement of the 
right half of the medulla oblongata and of the cervical cord extending to the 
eighth cervical segment. There was some enlargement from this point to the 
sixth dorsal ; below this the cord appeared to be normal. In the upper cer- 
vical segments the nervous structures of the spinal cord were replaced by a 
new growth ; in the lower cervical region the pressure effects of the new 
growth were evident. At about the level of the seventh cervical segment 
the central canal was lined with an unbroken layer of columnar ependymal 
cells, except at the posterior wall, which was broken through by a mass of 
larger glia cells, which partially filled the canal. (Fig. 77.) The glioma in- 
vaded nearly the entire cross-section of the cord, but undoubtedly originated 
in the vicinity of the central canal. 

The case is not unlike one reported by K. Miura ; Sokoloff described a 
glioma of the medulla, with cavity formation, in a boy, aged five years. 



CHAPTER XX. 

FAMILY DISEASES OF THE SPINAL OR CEREBRO-SPINAL 

SYSTEM. 

Family affections of the entire central nervous system 
constitute an important group of diseases. They are the re- 
sult of defective development, or possibly of early degener- 
ation of various parts and tracts in the brain and spinal cord. 
Of these disorders hereditary ataxy, or Friedreich's disease, 
is the best known. A few years ago Nonne and Marie de- 
scribed a type of disease which bears a close resemblance 
to hereditary ataxy, and for which Marie proposed the 
term Hereditary cerebellar ataxy (Heredo-ataxie cerebel- 
leuse). In Friedreich's disease the morbid process is re- 
stricted largely to the posterior or to the postero-lateral col- 
umns ; in Marie's disease there is in all probability a defec- 
tive development of the cerebellum and of the tracts con- 
nected with it. Congenital or hereditary defect of the 
pyramidal tracts also leads to another distinct group of dis- 
eases which we may term Hereditary spastic paralysis ; and 
Hoffman has still more recently described an hereditary 
form of progressive muscular atrophy of spinal origin in 
which the chief defect was found in the anterior gray mat- 
ter of the cord. These hereditary diseases suggest a clinical 
relationship to syphilitic disease of the spinal cord (which 
may be inherited, though not hereditary) and to some forms 
of cerebral birth palsies. Moreover, some of these hered- 
itary affections are distinctly cerebro-spinal, both as regards 
the distribution of the lesions and the character of the symp- 
toms. It is no easy task to separate these various forms 
from one another and to bring order out of chaos. The 
family form of progressive muscular atrophy will be con- 
sidered to much greater advantage in connection with the 
other forms of progressive muscular disease. 



334 



THE NERVOUS DISEASES OF CHILDREN. 



HEREDITARY ATAXY. — FRIEDREICH'S DISEASE. 

Hereditary ataxy, or Friedreich's disease, as it is called 
in honor of the physician who gave the first satisfactory ac- 
count of it, is a family af- 
fection. Several genera- 
tions are often affected by 
it ; but naturally we may 
find every now and then 
that the disease occurs 
in one or several mem- 
bers of a family without 
any history of a similar 
trouble in the patient's 
ancestry. It is not nec- 
essary, therefore, to speak 
of such cases as non- 
hereditary forms of Fried- 
reich's ataxia, as an Eng- 
lish writer has recently 
done, for the disease must 
invariably have a starting- 
point, and the patients 
under observation may 
indeed be the first of a 
long series that are to 
follow. The children who 
have come under my spe- 
cial notice in three dif- 
ferent families gave no 
history of any similar 
trouble in preceding gen- 
erations. 

The marked tenden- 
cy to the occurrence of 
this disease in families is 
shown, as Gowers states, 
by the occurrence of sixty-five cases in nineteen families, 
giving an average of rather more than three to each fam- 




Fig. 78.— Case of Hereditary Ataxy (Fried- 
reich's Disease). 



HEREDITARY DISEASES OF THE SPINAL CORD. 



335 



ily. The number has varied from two to as many as eight 
in a single family. Both sexes are about equally liable, but 
sometimes a special predilection is shown in favor of one 
or the other sex. In one family of nineteen, two males were 
affected with the disease and seventeen females escaped. 
All the cases which I have observed were in boys, the sis- 
ters of these escaping entire- 
ly unharmed. The disease 
comes on, as a rule, very early 
in life, most of the symptoms 
being fully developed before 
the age of fourteen. In the 
family referred to by Everett 
Smith, of Boston, the symp- 
toms of ataxic paraplegia 
were observed in the father 
at the age of sixty-six, but it is 
questionable whether his was 
a typical hereditary ataxy, 
and it is safer to adhere to 
the belief that Friedreich's 
disease will always appear 
early in life. If several mem- 
bers of one family are affected 
the disease is developed in 
all at about the same age. 

Symptoms. — The symp- 
tomatology of hereditary at- 
axy is now as firmly estab- 
lished as that of almost any 
spinal disease. Friedreich 
described most of the symp- 
toms in 1861. The French 
school, including Charcot, 
Vulpian, Brissaud, and Ma- 
rie, have given careful study to this disease. 
Carpenter, Gowers, Ormerod, and Bury described cases of 
true hereditary ataxy ; while in this country the chief con- 
tributions to this subject have been made by Smith, Ham- 
mond, Seguin, Dana, Church, Sanger Brown, and Inglis. 




Fig. 79. — Same Patient as in Fig. 78. 
Marked atrophy of the muscles about 
the shoulder girdles (mfra-spinati, su- 
pra-spinati, rhomboids, and others). 



In England, 



336 THE NERVOUS DISEASES OF CHILDREN. 

At a very early age children suffering from Friedreich's 
disease exhibit peculiarities in walking and standing. The 
child walks with its legs widely apart, in an uncertain, hes- 
itating fashion, reminding one very strongly of a combined 
ataxic and cerebellar gait. There are in addition in some pa- 
tients oscillatory movements of the head, which remind one 
a little of multiple sclerosis, and a little of such movements 
as we sometimes see in cases of senile degeneration. When 
the patient is asked to stand still, with his feet closely ap- 
proximated, he soon begins to stagger, as tabic patients do, 
and as soon as his eyes are closed falls to the ground un- 
less properly supported. Marie states that Romberg's 
symptom is ordinarily absent, but it has been most distinct- 
ly present in those subjects whom I have had occasion to 
examine. In addition to these disturbances in gait and in 
standing we also find a coarse tremor present in many of the 
cases, and in some a condition which is not so distinctly 
tremulous as it is ataxic and awkward. If the patient is 
asked to take hold of a pencil, to raise a glass of water to 
his lips, or to attempt to write, he seizes upon the object 
that he is to hold in a distinctly ataxic way and uses the 
hands awkwardly, sometimes after the fashion of extremely 
choreic movements. It is this combination of tremor, of 
awkward movements, and of choreic manifestations that 
gives to these children their characteristic aspect. There 
has been some discussion as to the occurrence of actual pa- 
ralysis ; in those whom I have examined there was in the 
first few years only a very slight loss of muscular power 
both in the upper and in the lower extremities. In ad- 
vanced stages the grasp is almost nil, and the lower extrem- 
ities may be so thoroughly paralyzed that it is impossible 
for the patient to lift a leg or even to move a toe, but in this 
respect the disease evidently varies very much. 

Sensation may be interfered with to a slight degree, as is 
maintained by Senator, Oppenheim, Rutimeyer, Soca, and 
others. Lightning pains are infrequent, although Charcot in- 
sists that in some instances these occur quite as typically as 
in tabes. Anaesthesia and analgesia are rarely present. Sev- 
eral French authors have described cases in which hemi- 
anaesthesia occurred; but Marie is inclined to attribute this 



HEREDITARY DISEASES OF THE SPINAL CORD. 337 

to the association of hysteria with Friedreich's disease. The 
muscular sense is rarely affected, and in the persons whom 
I have examined for the purpose of determining the pres- 
ence or absence of this sense, I have found it invariably 
present, even in those in whom there was marked inco- 
ordination of the upper extremities. 

Reflexes. — The superficial reflexes are, as a rule, pre- 
served ; but the deep reflexes are generally absent. The 
Babinski reflex is often present. The absence of the knee- 
jerks brings out in some subjects the very closest resem- 
blance between Friedreich's disease and tabes dorsalis. In 
cases in which the reflexes are exaggerated there is good 
reason to doubt whether the disease corresponds to that 
described by Friedreich;* but there is no reason why in 
those forms in which the affection involves both the poste- 
rior and lateral columns the knee-jerk should not occasion- 
ally be augmented, and it is probably in view of such mixed 
types that Gowers has spoken of a form of hereditary ataxic 
paraplegia under the heading of Friedreich's disease. It is 
one thing to maintain a type as originally described by its 
discoverer, and quite another thing to deny that any de- 
parture from such a typical series of symptoms implies an 
entirely different order of disease. 

Difficulties of micturition and of defecation occasionally 
occur, but are not so constant an accompaniment of Fried- 
reich's disease as they are of tabes. Profuse salivation 
and disturbances in respiration (Oppenheim) have been 
recorded. 

Trophic and vasomotor disturbances are also rare, if we 
except a general discoloration of the skin and coldness of 
feet and hands. Marie and other French authors refer to 
the occurrence of a special form of club-foot which they 
have observed in these cases of hereditary ataxy. Marie 
states that the foot is shortened ; that the anterior portion 
is particularly broad if viewed laterally ; the foot is in the 
condition of a pes cavus. The toes are hyperextended and 
have a claw shape. This deformity of the toes is said to 
have been observed as one of the early symptoms by parents 
in whose families this special disease has been hereditary. 

* Some of these may belong to the Heredo-ataxie cerebelleuse of Marie. 



33S 



THE NERVOUS DISEASES OF CHILDREN. 



Muscular atrophy constitutes an important symptom of 
this disease. It is most distinctly visible in the shoulder 
and pelvic girdles. It was so prominent a feature in one of 
my patients that on first examination I was inclined to re- 
gard the trouble as one of an hereditary form of progressive 
muscular atrophy, until the examination of the brother, who 
was in a more advanced stage of the disease, proved to me 
beyond the possibility of a doubt that both cases were typ- 
ical of Friedreich's disease. In the boy represented in Fig. 
90, the excessive atrophy must be attributed to an addi- 
tional involvement of the gray matter of the cord. 




Fig. 80. — Deformity of the Feet in a Case of Friedreich's Disease ; Hyperextension 
of the Toes and Club Foot. (Marie.) 



We have now to consider a further set of symptoms 
which seem to me to prove the cerebro-spinal character of 
the disease. Nystagmus occurs in many of these cases, but, 
as a rule, does not appear until several years after the onset 
of the first symptoms. The nystagmus can often be elicited 
only upon extreme use of the ocular muscles. In this con- 
nection it may be well to remember that nystagmus upon 
extreme movements occurs in not a few healthy individuals, 
and that the presence of such movements in a child of a 
family affected with Friedreich's disease need not neces- 
sarily indicate the development of this serious trouble. 
Ocular palsies occur, but are rare. The Argyll-Robertson 



HEREDITARY DISEASES OF THE SPINAL CORD. 339 

pupil is as regularly absent in Friedreich's disease as it is 
present in cases of tabes. Optic atrophy and amblyopia 
have not been described in these cases. All the other 
senses remain entirely unaffected. 

Among cerebral disorders occurring in the course of 
Friedreich's disease, vertigo and dizziness are extremely 
frequent. These may in part be due to the oscillatory 
movements of the head. In the earlier years of the disease 
the intelligence is not affected, except that the children can- 
not be educated at schools as other children are, and there- 
fore remain backward. As the disease progresses a distinct 
defect of intelligence is noticeable, and a condition of semi- 
idiocy may be the result. The patients appear, however, 
far more idiotic than they really are, and this appearance is 
caused by the peculiar stupid expression of face, and above 
all by the disturbances in speech, to which we shall now 
refer. The speech reminds one a little of multiple sclerosis 
in that it is slightly scanning, but it is at times hesitating 
and jerky, at other times slow, deliberate, and awkward. 
On the whole, I find Marie's simile a good one, who com- 
pares it with the cerebellar gait, since it is, as he says, awk- 
ward, uncertain, and vacillating. All these symptoms are 
not developed until after the lapse of years. At first the 
peculiar gait and position are noticeable, later on the inco- 
ordination of the upper extremities and the difficulties of 
speech become more marked ; then, after the lapse of a 
longer or shorter period of time, the patients become help- 
less and may .remain bedridden or confined to their chairs 
for a period of ten and even twenty years. 

Differential Diagnosis. — Attention must be directed 
to the following points : 

Tabes. Friedreich's Disease. 

Argyll-Robertson pupil present. Argyll-Robertson pupil absent. 

No nystagmus. Nystagmus present in later years. 

No tremor of the head. Tremor of the head present. 

No peculiarities of speech. Speech altered. 

Inco-ordination of upper extremities Inco-ordination the rule. 

rare. 

Lightning pains and various crises. No such pains and crises, as a rule. 

Ataxic gait. Ataxic-cerebellar gait. 

No defect of intelligence. Intelligence defective in later years. 



340 THE NERVOUS DISEASES 0E CHILDREN. 

In the above parallel columns the symptoms which both 
have in common have been omitted, such as absence of re- 
flexes and Romberg's symptom. 

Multiple sclerosis can be distinguished easily from 
Friedreich's disease by the excessive exaggeration of the 
reflexes, by the intention tremor, by the marked spasticity 
of the gait, by the occurrence of ocular palsies, all of which 
rarely occur in cases of Friedreich's disease. Confusion 
might arise from consideration of the similarity in speech 
and the occurrence of nystagmus. Marie includes in the 
consideration of the differential diagnosis ordinary chorea, 
but it seems to me that no one but a very obtuse person 
could be misled by the occasional occurrence of awkward 
and choreic movements in Friedreich's disease, for in no 
other respect are the two diseases at all similar. 

Confusion may more readily occur with cerebellar diseases, and Schultze 
some time ago, in answer to Senator's observations, called attention to the 
fact that, after all, cases that would seem to be of the cerebellar order are 
altogether different from the type which Friedreich described. I can ap- 
prove of this remark of Schultze, for I have myself seen a case of defective 
cerebellar development* in which there was a superficial resemblance be- 
tween it and cases of Friedreich's disease, and yet a careful considera- 
tion of the conditions was sufficient to distinguish between the two sets of 
diseases. 

Pathological Anatomy. — The morbid anatomy of 
Friedreich's disease has not, as yet, been satisfactorily de- 
termined. Many autopsies have been made, but in some of 
the cases which have been examined post mortem, the symp- 
toms have varied so much from the type, as laid down by 
Friedreich, that the results of the examination have helped 
to obscure rather than to clear up the doubts regarding the 
true anatomical changes of the disease. A number of au- 
thors have called attention to the attenuation of the entire 
cord in cases of hereditary ataxia, the diameter of the cord 
being but three-fourths or two-thirds of the normal. This 
attenuation is most marked in the dorsal region ; but 
whether it is due to a disappearance of nerve-fibres, to the 
retraction of sclerotic tissue, or whether it is simply a de- 
fect of development, has not been clearly made out. Any 

* See Chapter on Conditions due to Defective Development of the Brain. 



HEREDITARY DISEASES OF THE SPINAL CORD. 



S4i 



one of these three causes may be operative in some cases, 
or a co-operation of all three is a possibility in others. The 
one fact that is absolutely indisputable is that on micro- 
scopical examination a sclerosis of the spinal cord is found, 
involving at different levels, or at one and the same level, 
various systems of the cord. This sclerosis affects most 
frequently the posterior columns or the lateral columns, or 
both together, and hence the symptoms vary between those 
of a pure posterior spinal sclerosis and those due to a 




Fig. 8i.— Section through Middle Dorsal Region of a Case of Friedreich's Disease. 
(After Blocq et Marinesco, from Marie's treatise.) A, small degenerated area, prob- 
ably Gowers' tract ; B, degeneration in lateral columns ; C, direct cerebellar tract ; 
D, column of Burdach ; E, column of Goll — both degenerated ; G, a strand of 
healthy fibres. 



postero-lateral sclerosis, resembling the symptoms of the 
ataxic paraplegia of the adult. 

Taking up the lesions of Friedreich's disease in detail, 
we may note the following distribution : 

i. In the posterior columns, in a number of the cases, the columns of 
Goll are sclerosed throughout their entire length from the lower part of the 
spinal cord to the calamus scriptorius. The column of Burdach is also in- 
volved throughout the greater part of its course ; but, according to Marie, the 
intensity of this sclerosis varies in different levels of the cord. The external 
portion of the column of Burdach, near the posterior horns, is generally ex- 
empt from sclerosis ; in the cervical region the sclerosis of the column of 
Burdach begins to diminish, and disappears altogether in the vicinity of the 
medulla oblongata. 



342 



THE NERVOUS DISEASES OF CHILDREN. 



2. The direct cerebellar tract is involved from its beginning in the lower 
dorsal region to the upper cervical region. Toward this latter part it dimin- 
ishes considerably, but is most marked in the upper dorsal segments. Sev- 
eral observers have noted that the lesion extends beyond the direct cerebellar 
tract, and involves the antero- lateral tract, or tract of Gowers ; and Marie 
goes to the extent of stating that he thinks this involvement of the antero- 
lateral tract an almost constant feature of Friedreich's disease. 

3. As for the lateral columns, the opinions of authors differ somewhat. 
Marie is not willing to allow the regular involvement of the lateral columns, 
while he concedes that the diseased fibres occupy the position of the crossed 
pyramidal tract. He does not believe that they represent the fibres of that 




Fig. 82 — Section through a Cervical Segment from a Case of Friedreich's Disease. 
(Schultze.) Degenerated areas in posterior and lateral columns and in anterior col- 
umn (left half of figure). Posterior root fibres also degenerated. 



tract. His reasons for maintaining this are, first, that the lesion of the lateral 
columns in Friedreich's disease diminishes considerably from below upward to 
the level of the lower portion of the medulla oblongata, the very reverse of 
which would occur if these fibres were part of the pyramidal tract. Secondly, 
that on a transverse section of the spinal cord the localization of the lesion 
of the lateral column does not correspond exactly to the site of the lesion of 
the pyramidal tract proper. Third, nothing in the clinical appearances of Fried- 
reich's disease reminds one of the symptoms which are a constant accom- 
paniment of changes in the pyramidal tract. The only explanation which 
Marie is able to give for the fibres that are affected in the lateral region is 
that they are probably fibres which connect the direct cerebellar tract with the 
antero-lateral tract of Gowers. Of the reasons advanced by Marie, the first 
seems to me to be the only one that will bear close scrutiny ; the second 
is scarcely susceptible of proof, and as for the third, it is sufficient to state 



HEREDITARY DISEASES OF THE SPINAL CORD. 343 

that the disease attacks both the posterior columns and the pyramidal fibres 
at various levels. 

4. A difference of opinion also exists as regards the marginal zone of 
Lissauer, as many authors claim to have found these zones entirely intact, 
while others claim that an affection of these fibres constitutes a regular feat- 
ure of Friedreich's disease. At all events the affection of Lissauer's tract is 
not nearly so constant or so early a feature of the disease as it is in the spinal 
ataxia in the adult. 

The morbid anatomy of Friedreich's disease is not exhausted with this 
statement regarding the behavior of the white fibres. The gray matter is 
affected as well. The columns of Clarke exhibit a loss of fibres very much 
like that in tabes, as well as a considerable diminution of their cells, which 
are also smaller and devoid of their long-cell processes. In the posterior 
horns there is a diminution of volume, and also a decided diminution in the 
number of cells. The anterior horns have been found altered by some, 
Friedreich being among them, and the cells may be atrophied. There is 
no reason to doubt this occurrence in view of a considerable atrophy of the 
muscles which occurs in some of these cases, as in one of my own. 

Some changes have been noticed in the ependyma of the central canal and 
in the cerebellum (Barker); yet these are not regular morbid features of 
Friedreich's disease. Some have also insisted that the spinal meninges are 
involved, while others dispute such involvement. If they are affected at all 
it is in the vicinity of the posterior columns. Little more can be said of the 
behavior of the posterior roots, although Blocq and Marinesco insist that they 
are as frequently altered in Friedreich's disease as they are in ordinary tabes. 
The peripheral nerves are supposed to be entirely normal in Friedreich's dis- 
ease, and to this fact Dejerine and others attributed the entire absence of 
fulgurating pains in hereditary ataxy, while they are universally present in 
tabes dorsalis. 

Senator laid especial stress on the congenital atrophy of the cerebellum 
and considered it to be of greater importance than all the spinal cord 
changes. 

The question remains as to the nature of the morbid proc- 
ess underlying Friedreich's disease? Dejerine and Letulle, 
on the strength of certain peculiar fibres which they have 
found, and which they consider similar to fibres discovered 
by them in the cortex of epileptic patients, are inclined to 
regard the sclerosis of hereditary ataxia as a sclerosis of the 
neuroglia. They insist that neither the connective-tissue 
septa nor the blood-vessels are at all altered, and that the scle- 
rosis is therefore very different from the vascular sclerosis 
which so often involves the pyramidal tracts and the direct 
cerebellar tract ; but later investigators, among them Wei- 
gert, have disproved these views of Dejerine and Letulle. 



344 THE NERVOUS DISEASES OF CHILDREN. 

They regard the sclerosis of Friedreich's disease as in no- 
wise different from the ordinary sclerosis excepting in this, 
that it has been developed at an unusually early period. 
Weigert, in his recent studies on the neuroglia, concludes 
that in the ordinary spinal sclerosis, as it occurs in tabes, in 
multiple cerebro-spinal sclerosis, and in amyotrophic lateral 
sclerosis, the proliferation of the neuroglia is a much more 
marked feature than in hereditary ataxy. The refutation 
of this theory of Dejerine and Letulle does not, how- 
ever, help us in the recognition of any true theory of the 
disease, and for the present we must confess that we do not 
know why or how the sclerosis of Friedreich's disease is 
developed. 

Prognosis. — The prognosis of Friedreich's disease is in- 
variably grave. In the course of four to six years the patient 
becomes crippled, unable to move about except in a chair, 
awkward in speech, and more or less demented. Unfortu- 
nately the disease does not lead to a fatal issue within a 
reasonable period of time, some of the patients attaining the 
age of forty years and more. 

As for treatment, no special method need be recom- 
mended. The progress of the disease cannot be stayed, the 
relief of pain constituting the only reason for active inter- 
ference on the part of the physician. The ordinary anti- 
neuralgic remedies may be employed to this end. In addi- 
tion, suspension, electrical treatment, massage, and the like, 
may be ordered, in the discretion of the physician. 

HEREDITARY ATAXY (CEREBELLAR TYPE ; TYPE NONNE-MARIE). 

Under the title " Heredo-ataxie Cerebelleuse " Marie collected a number 
of cases which bear a very close resemblance to Friedreich's disease, yet pre- 
sent several characteristic and different symptoms. Titubation, Romberg's 
symptom, tremor of the head and of the extremities, choreiform, awkward 
movements, particularly of the arms and hands, and nystagmus, are symp- 
toms common to Friedreich's disease, and to the form we are now describing. 
The cerebellar form of hereditary ataxy comes on, as a rule, at the age of pu- 
berty or later. The knee-jerks, instead of being diminished, are increased. 
There are marked ocular symptoms, the pupils fail to react to light and dur- 
ing accommodation. There is diplopia in some cases, color-blindness in 
others. Contraction of the visual field has been observed, and amblyopia, due 
to atrophy of the optic nerve, is a typical symptom. There are also marked 



HEREDITARY DISEASES OF THE SPINAL CORD. 345 

disturbances of sensation. The deformities of the foot and kyphosis, noted in 
Friedreich's disease, are never present in the cerebellar type. 

This symptomatology, as developed by Marie, reveals a very striking re- 
semblance between these cases of hereditary cerebellar ataxy and a form of 
family disease described by Nonne. In all probability Nonne's and Marie's 
descriptions refer to the same type. While the latter deserves credit for 
naming the disease, it seems to me just that Nonne's name should be con- 
nected with this type. There is also a very close resemblance between this 
hereditary cerebellar ataxy and some of the cases which have been described 
under the heading of " Hereditary Spastic Paralysis." Senator's views regard- 
ing Friedreich's disease may in part be explained by the recognition of this spe- 
cial order of disease, and there would surely be a possibility of confounding 
this type of hereditary ataxy with the cerebellar type of multiple sclerosis 
which Charcot has described. 

It is too early to report definitely upon the pathology of hereditary cere- 
bellar ataxy. If we are correct in considering the cases described by Marie 
and Nonne as representatives of the same type, the post-mortem findings in 
one of Nonne's cases will give some clew to the pathology of this variety of 
disease. In Nonne's case the cerebellum was small, and there was a simple 
diminution of tissue without sclerosis or degeneration. The spinal cord was 
much attenuated throughout its entire length. Under the heading of " He- 
reditary Ataxy and Atrophy of the Cerebellum " Menzel described a condition 
in which there was a combined systemic disease of the spinal cord, and in 
addition atrophy of the cerebellum, pons, and medulla. Two French authors, 
Royet and Collet, have reported a case which was diagnosticated during life 
as multiple sclerosis, in which there was found on post-mortem examination 
a lesion of the cerebellum and of the cerebellar tracts in the pons. It is 
possible that some of the cases which have been regarded as a form of he- 
reditary multiple sclerosis may come under this same heading. I recognize 
that there is great danger in multiplying types of disease. The form de- 
scribed by Marie and Nonne should in justice to these two authors be given 
due consideration ; but I believe it better for the present to regard it as a 
variety of hereditary ataxy.* 



HEREDITARY SPASTIC PARALYSIS (SPINAL, CEREBRAL OR 
CEREBRO-SPINAL TYPES). 

During the past decade or two several family affections 
have been described by Homen, Newmark, Pelizaeus, Strum- 
pell, Freud, Erb, and myself, in all of which a spastic 
rigidity, or a spastic paralysis affecting the lower extremi- 
ties chiefly, has been the most prominent symptom. In 
some cases the spastic symptoms were evidently of purely 

* The cases reported by Dr. Sanger Brown also resemble this type. 



346 THE NERVOUS DISEASES OF CHILDREN. 

spinal origin ; in other cases they were associated with dis- 
tinct cerebral symptoms. A classification of all of these 
various diseases according to the morbid conditions under- 
lying them, is not yet possible ; it is sufficient for the pres- 
ent to discuss them from the etiological point of view. I 
have chosen, therefore, to designate them as hereditary 
spastic paralysis, and to subdivide them, according to their 
more prominent symptoms, into a spinal, and a cerebral or 
a cerebro-spinal variety. 

After adopting a subdivision of this kind, the pathology 
of each type will be more readily made out ; and it is quite 
probable that the various types will be found to represent 
a mere difference in the topographical distribution of le- 
sions and symptoms, while the morbid process, an arrest of 
development, or an early degeneration, is common to all. 

The spinal type of hereditary spastic paralysis is well 
illustrated by Newmark's cases. In view of the impor- 
tance of the subject a short summary of a few of his cases 
will be given. 

A girl, aged fifteen, tall and intelligent ; normal birth and labor ; no con- 
vulsions. Did not kick with the legs as other children do in bed. Began to 
walk at eighteen months. Gait peculiar from the first, and always remained 
typical of spastic paraplegia. Lower extremities flexed at the hips and 
knees ; adduction of thighs and pes equinus. No wasting of muscles, which 
react well to both currents. Knee-jerks and adductor reflex exaggerated ; 
no ankle clonus (probably on account of extreme contracture). No disturb- 
ance in the upper extremities, but all tendon reflexes exaggerated in them. 
Jaw-jerk present. Intelligence, sensations, and sphincters intact. No nys- 
tagmus, no strabismus. Girl is able to use a tricycle. Speech is normal. 
A brother, aged five, exhibits very similar symptoms. No history of syphilis 
in the family. There were no other cases of similar nature in the family, ex- 
cept one of spastic diplegia in a first cousin ; but this diplegia was evidently 
the result of difficult labor. 

In the second family the father is said to be a tall, healthy man of thirty- 
eight, who denies syphilitic infection or alcoholic habit. Has never known 
of any affection similar to that observed among his children. His reflexes 
are normal. Mother of children, aged thirty-seven, has always been healthy. 
Her knee-jerks and Achilles tendon reflex quite active. Lively reflexes in 
the upper extremities and jaw-jerk distinct ; no ankle clonus. No blood re- 
lationship between man and wife. They have had eleven children, eight 
of whom are living. The oldest living child is a boy, aged sixteen, who 
was well until about a year and a half before he was examined. At that 



HEREDITARY DISEASES OF THE SPINA I CORD. 347 

time a stiffness appeared in the legs, especially on rising in the morning. 
His condition gradually developed into a mild form of spastic paraplegia, 
with typical gait and position of the extremities. Knee-jerks much exag- 
gerated ; no patellar clonus nor ankle clonus. Plantar, cremasteric, and 
abdominal reflexes very lively. Abdominal muscles said to be rigid. Up- 
per limbs normal ; reflexes much increased. Jaw-jerk well marked. In a 
second brother, aged fourteen, the symptoms had become more pronounced, 
and had set in at the age of seven and a half years. The legs were stiff and 
walking was difficult ; had been compelled to use crutches. There were con- 
tractures in the knee-joints ; there was adductor spasm and pes equinus, 
furthermore, an exaggeration of the knee-jerks. Ankle clonus was present, 
and the plantar and cremasteric reflexes were also very lively. A third 
brother developed exactly the same symptoms at the age of nine years, after 
typho-malarial fever. Though these three cases are the most pronounced 
ones in the family, one sister exhibits increased reflexes ; a boy, eight years 
of age, is said to be stiff in the knees, with exaggeration of the patellar re- 
flex ; another child, aged six, is said to have a lazy, dragging walk, with ex- 
cessive increase of knee-jerks ; a child, aged three and one half, has active 
knee-jerk and jaw-jerk. 

That there is a tendency to spastic paralysis in this 
family there is no doubt ; but the fact that instruments were 
used at the birth of several children, including the one whose 
symptoms were most pronounced, makes it probable that 
" heredity " alone was not responsible for the multiplicity 
of cases. In a more recent publication (1904) Newmark 
reports upon the development of the children referred to in 
his earlier articles. 

The symptoms of this spinal type (spastic paraplegia, 
with rigidity or contractures, increased reflexes) are 
distinctly due to interference with the pyramidal tracts 
in the lateral columns of the cord. Many years ago 
Strumpell described the cases of two brothers, who pre- 
sented the typical features of a spastic spinal palsy. In 
one of these cases a post-mortem examination was made, 
by which it was determined that the symptoms were due 
to a primary systematic degeneration of both pyramidal 
tracts in the lateral columns, together with a slight affec- 
tion of the cerebellar tracts and the columns of Goll. More 
recently Strumpell has described another case, very much 
like the other two, in which he made the diagnosis of he- 
reditary spastic palsy. In the family of this patient the 
grandfather was said to have suffered from a palsy of the 



348 THE NERVOUS DISEASES OF CHILDREN. 

legs, the father had a peculiar gait, and people who knew 
both the father and the present patient said that one walked 
like the other. Two uncles w T ere said to have the same walk. 
The mother of the patient and other brothers and sisters 
were entirely healthy. The patient did military service 
from his twenty-first to his thirty-third year, and was not 
inconvenienced in any way, except that he occasionally 
noticed a peculiar feeling in the legs. At the age of about 
thirty-five his first symptoms began. His legs became stiff 
and he soon had to resort to a stick in walking. The exam- 
ination of the patient revealed a typical spinal spastic 
paralysis, with exaggeration of the reflexes, rigidity of the 
joints, normal sensation, and a spastic gait. The vesical and 
rectal reflexes were not interfered with. All the symptoms 
steadily increased, but no new ones were developed. In 
addition to its interest as a form of hereditary spinal disease 
the type also deserves some special recognition from the 
fact that, from first to last, the symptoms have been such 
as are due exclusively to disease of the lateral columns. 
Striimpell refers to Bernhardt's patients, and claims that 
they are entirely identical with those described by him. 
Four out of six brothers presented the phenomena of spastic 
spinal palsy ; three of these four developed their symptoms 
at the age of thirty, and all the morbid signs progressed in a 
remarkably slow fashion. I can agree with Striimpell in 
believing that these diseases are entirely similar to those de- 
scribed by him, although in one case of Bernhardt the symp- 
toms tended toward a cerebro-spinal rather than a purely 
spinal type. The forms described by Tooth and Philip 
should be placed in the same category. 

Spastic spinal paralysis (spastic paraplegia) occurs in children of families 
in which there is no history of a similar affection in the same or past genera- 
tions. In February, 1893, a girl, four years of age, was brought to my clinic, 
who was afflicted with a typical spastic paraplegia ; paresis and rigidity of 
both lower extremities ; very rigid gait, increased knee-jerks on both sides, 
slight double ankle clonus — these reflexes being a little more marked on the 
right side than on the left side; increased reflexes in the upper extremities, but 
no weakness. Electrical reaction of all muscles and nerves normal ; no dis- 
turbances of sensation ; no impairment of vesical and rectal reflexes ; no 
nystagmus. Intelligence good. The birth of the child (first-born) had been 
entirely normal and at full term ; no instruments were used ; the child was 



HEREDITARY DISEASES OF THE SPINAL CORD. 349 

supposed to be perfectly well, except that it did not begin to walk until it was 
three years old, and its walk was unusually stiff and awkward ; it dragged its 
feet. Three younger children are perfectly healthy. Excepting the early age 
of onset there is no distinction in clinical symptoms between such cases as 
these and the spastic spinal paralysis of the adult. 

Morbid Anatomy. — In one of Newmark's cases there 
was a distinct degeneration of the pyramidal tracts, most 
marked in the lumbar portion of the cord ; a degeneration 
of the posterior columns, most marked in the upper dorsal 
region ; also an involvement of the columns of Clarke — in 
short, a combined degeneration not unlike the findings in 
Strumpell's cases and in Friedreich's ataxia. In view of 
Strumpell's investigations there is no longer any doubt 
about the occurrence of a primary degeneration of the 
lateral columns developing later in life, but due in all proba- 
bility to abnormal congenital conditions. There is some 
reason, therefore, to believe that the question of the occur- 
rence of a primary lateral sclerosis will be affirmatively an- 
swered through these studies on hereditary spastic paralysis. 

Diagnosis. — The diagnosis of these diseases need not 
be given in further detail. The history of a case exhibiting 
the symptoms of spastic paraplegia of the lower extremities, 
with increase of the reflexes, with rigidities and contrac- 
tures, without involvement of the vesical or rectal reflexes, 
without atrophy, without disturbances of speech and nys- 
tagmus, would be sufficient to place the case in this cate- 
gory, provided the hereditary character of the affection 
could be established. The disease should be carefully dif- 
ferentiated from the spastic cerebral palsies of childhood.* 
The diplegias and paraplegias occurring as a result of diffi- 

* In an article on Hereditary Spastic Paralysis, Erb stated that some of the con- 
genital diplegias and paraplegias which Freud, myself, and others have attributed to 
cerebral lesions may at times be due to spinal lesions. I am willing to concede the 
possibility of this ; but I think that such spinal affections are, after all, rare ; and there 
is the one fact, to which I have alluded in various writings, that in forty-five per cent, 
of all the cases of infantile spastic paraplegia there is marked idiocy. This association 
militates against the diagnosis of a purely spinal disease. I have reviewed the his- 
tories of all my patients with supposed cerebral paraplegia and can find none which I 
am willing to attribute to a spinal lesion. The child reported on page 348, though suf- 
fering from paraplegia, was recognized as probably of spinal origin ; and such an 
origin may be suspected in types of spastic paraplegia, without idiocy coming on 
several years after a normal birth. 



350 THE NERVOUS DISEASES OF CHILDREN. 

culty during labor (Little's disease), would be most easily 
confounded with this hereditary form. A careful inquiry 
into the history of labor, the determination of the exact age 
at which the trouble began, the frequent occurrence of con- 
vulsions and of defective mental development, in birth 
palsies, will help to differentiate between these two sets of 
cases. If spastic paraplegia is developed in a child, or in a 
youth, that has not been preceded by convulsions, or is not 
associated with defective mental development, the case 
would come more properly under the head of hereditary 
spastic paraplegia, and we must remember that such cases 
may occur without any history of similar disease in the same 
or preceding generations.* 

The prognosis of hereditary spastic spinal paralysis is 
a grave one as regards recovery from the disease, but not 
unfavorable as regards the duration of life, for the majority 
of patients thus far examined have been well advanced in 
years. In the way of treatment, nothing can be attempted 
except to apply the usual methods of massage and elec- 
tricity, or possibly to attempt to improve contractured 
limbs by various tenotomies and by transplantation of 
tendons. 

CONGENITAL SPASTIC PARAPLEGIA. (LITTLE'S DISEASE.) 

In this connection brief reference must be made to an affection which is 
generally spoken of as " Little's Disease." It refers to a condition of spastic 
rigidity of the limbs .with paralysis which is observed in the earliest months 
and years of life, and is almost invariably due either to defective development 
or to injuries received during protracted labor. Van Gehuchten, without 
sufficient justification, restricts the term to the disease occurring in children 
born before full term. As the present author showed in 1897, so many 
different conditions have been collated under the term Little 's Disease that 
much confusion has resulted. It will be well, therefore, to quote from 
Little's original paper in " The Transactions of the London Obstetrical 

* Marie and some other French authors deny the existence of a primary spinal 
spastic paralysis in the adult, and believe that if such a condition does exist, it is to be 
traced back to the early years of life. They describe as " Tabes dorsal spasmodique " 
of children, a group of symptoms which German and American authors have included 
under the term, Congenital Spastic Diplegia and Paraplegia. The French view would 
take all of these cases out of the category of cerebral diseases, and for this there is as 
yet no warrant. Marie (p. 101) states that "Tabes dorsal spasmodique" is never 
hereditary ; and attributes the condition to defective development of the pyramidal 
tract. This tract is surely a cerebro-spinal affair. 



HEREDITARY DISEASES OF THE SPINAL CORD. 35 1 

Society," Vol. 3, 1862, in which he says: "I showed that premature birth, 
difficult labors, mechanical injuries during parturition to head and neck 
where life has been saved, convulsions following the act of birth, were apt to 
be succeeded by a determinate affection of the limbs of the child which I 
designated, spastic rigidity of the limbs of new-born children, spastic rigidity 
from asphyxia neonatorum and assimilated it to the trismus nascentium 
and the universal spastic rigidity sometimes produced at later periods of 
existence." 

He speaks of spastic rigidity being sometimes associated with paralytic 
contraction. He evidently had a paralytic condition in mind, and in that 
respect various recent writers who seem to deny the existence of paralysis in 
these cases and wish to speak of them only as spastic rigidities, are in the 
wrong. 

In association with Dr. Peterson I showed many years ago that the 
frequent occurrence of mental defect with congenital spastic rigidity and 
paralysis was due to the various cerebral lesions and accidents which are 
responsible for other cerebral palsies of children. The symptoms are, as a 
rule, limited to the lower extremities, although on examination some slight 
difficulty in the upper extremities may frequently be noted. Cross-legged 
position and cross-legged gait are typical symptoms of this trouble. There 
is, of course, close resemblance to disease involving the lateral columns of 
the cord, and in this respect the cases must be carefully differentiated from 
primary or hereditary spastic paralysis and from syphilitic spinal paralysis. 

Recent observations of Dejerine and of Spiller have shown that con- 
genital spastic paraplegia may be due to changes in the lateral columns 
of the cord only, without any cerebral disease, but in spite of these two 
trustworthy findings, the present writer still believes that in most instances 
the conditions which could reasonably be designated as Little's Disease, are 
to be attributed to defective development of, or to lesions in, the brain. 
Further information on this subject can be gathered from the chapter on 
infantile cerebral palsies. It will be recognized that if the term Little's 
Disease is to be retained at all, it should be applied only to the cases of 
spastic paraplegia, or of spastic diplegia (complete or incomplete), due to 
premature birth or to difficulties during parturition. 



CHAPTER XXL 
PROGRESSIVE MUSCULAR ATROPHIES. 

In this chapter we intend to discuss all those diseases 
which are characterized by a progressive weakness and 
atrophy of certain groups of muscles. We have nothing 
to do with muscular atrophy, whether progressive or not, 
which follows after acute disease of the brain, of the spinal 
cord, or of the peripheral nerves. The term ''progressive 
muscular atrophy " was formerly given to a single type of 
disease with which we shall become more intimately ac- 
quainted, and for a time all cases resembling this one type 
were designated in the same Avay. It became evident, how- 
ever, that this one term was altogether too general. While 
it was a convenient clinical designation for an entire group 
of diseases, it did not sufficiently describe a number of 
other forms which were closely allied to the chief type. 
In order to avoid confusion, it would be well if we could 
dismiss the term progressive muscular atrophy altogether, 
for in many of the cases hypertrophy as well as atrophy is 
present for a long period of time. The word dystrophy, 
which might be used to designate both conditions, has, un- 
fortunately, been restricted to the cases of primary or idio- 
pathic muscular wasting ; we cannot, therefore, apply it to 
the spinal forms. Moreover, a number of diseases were 
included under the heading of progressive muscular atrophy 
which we now recognize to have been cases of amyotrophic 
lateral sclerosis, of syringomyelia, and possibly of spinal 
syphilis. 

The chief question at the present time regarding the 
various forms of progressive muscular atrophy is, whether 
in a given case the disease is of spinal or muscular origin. 



PROGRESSIVE MUSCULAR ATROPHIES. 



353 



Amyotrophy 



Neural Atrophy 



Those forms of progressive muscular atrophy due to a 
spinal lesion we call amyotrophies, and to those due to disease 
of the muscular system alone, we give the name myopathies. 
We shall see that there is some reason, too, to constitute a 
third type, which we might designate as a neural form of 
progressive muscular atrophy. (Fig. 83.) 

The study of this entire subject began many years ago 
with the establishment of two distinct diseases — the first was 
the typical "progressive muscu- 
lar atrophy," as described by 
Aran and Duchenne ; the sec- 
ond, pseudo-hypertrophic mus- 
cular paralysis. Since that 
time at least six different forms 
of progressive muscular wast- 
ing have been described, and 
the attempt has been made in 
each case to prove the relation 
of the special form, either to 
the Aran-Duchenne type, or to 
the type of muscular pseudo- 
hypertrophy. The Aran-Du- 
chenne type has become the 
chief exponent of progressive 
amyotrophies ; while muscular 
pseudo-hypertrophy has been 
considered the most pro- 
nounced form of primary myo- 
pathies. The various types of 
progressive muscular disease 
have been established very largely in accordance with 
the mere topographical distribution of atrophy or hyper- 
trophy. Though convenient for clinical designation, such 
a distinction is not sufficient for a rational classification 
of these various diseases. It must be our aim to find the 
cardinal symptoms which will help us to differentiate at 
once, and easily, between those cases of progressive mus- 
cular disease due to spinal-cord lesions and those primary 
dystrophies, which represent a disease of the muscular 
system. 




Myopathy 

Fig. 83 — A Diagram designed to show 
the Site of the Morbid Lesion in the 
Several Groups of Progressive Mus- 
cular Atrophy. 



354 THE NERVOUS DISEASES OF CHILDREN. 

The following are the cardinal symptoms present in the majority of cases 
belonging to the two large groups of cases : 



Progressive Amyotrophies. Progressive Myopathies. 

Onset late in life ; rarely in early Onset in early life. 

childhood. 

Not hereditary, as a rule. Generally hereditary (family trouble). 

Wasting first in the upper extremi- Wasting or hypertrophy begins in 

ties (leg type rare). the lower extremities. 

Hypertrophy does not occur. Hypertrophy frequent. 

Fibrillary twitchings. No fibrillary twitchings. 

Reaction of degeneration often pres- Reaction of degeneration rare (quan- 

ent in affected muscles. titative, not qualitative, electrical 

changes). 

These points of differential diagnosis will hold good in the majority of cases. 
Werdnig, Hoffmann, and others have reported cases of spinal progressive 
muscular disease due to hereditary or family influences. Fibrillary twitch- 
ing has been seen in some cases that appeared to be typical myopathies, 
and the electrical reactions have been found considerably altered in similar 
cases, so that of all these cardinal symptoms there are, after all, only a very 
few which are invariably present in one or the other form of progressive mus- 
cular disease, and it is wiser, therefore, to be guided by the general agree- 
ment of symptoms rather than by any one single symptom.* This confusion 
of types of disease and of symptoms, need not cause surprise, if we remem- 
ber that the ganglion cells of the spinal cord, the peripheral nerves, and the 
muscles, constitute a physiological unit (Neuron). 

With these prefatory remarks we may proceed to the 
consideration of that class of cases which for a very long- 
time were supposed to be the only representatives of what 
was formerly called "progressive muscular atrophy." This 
is a spinal-cord affection which, as a rule, begins late in life. 
It might, therefore, be considered out of place to treat of 
this disease in a work on the nervous diseases of children ; 
but the entire subject of muscular diseases cannot be prop- 
erly understood unless we can recognize this special type, 
and, furthermore, cases of this type have of late years been 
described by Hoffmann and others, in children. 

* The hereditary cases reported by Werdnig are particularly interesting in this re- 
spect. 



PROGRESSIVE MUSCULAR ATROPHIES. 355 

PROGRESSIVE AMYOTROPHY. — "PROGRESSIVE MUSCULAR 
ATROPHY." (TYPE, ARAN-DUCHENNE.) 

This form begins, in the majority of the cases, with an 
atrophy and a corresponding weakness in the small muscles 
of the hand (thenar and hypothenar). The atrophy extends 
slowly from muscle to muscle (" atrophie individuelle "), be- 
ginning as a rule with the adductor pollicis. It involves by 
degrees the opponens pollicis and the deep muscles of the 
thenar. From these it gradually extends to the muscles of 
the hypothenar, the interossei, the flexors and extensors in 
the forearm. At this point the disease may remain sta- 
tionary, or it may spread to the flexors in the upper arm, to 
the deltoid, the triceps, and finally to the muscles of the 
trunk, the shoulders, and the back. Duchenne recognized 
the fact that the atrophy may, in exceptional cases, begin in 
the trunk, in the shoulders, or in the legs. Some of these 
would now, no doubt, be considered under a different head- 
ing, but I have myself seen cases which could in nowise 
be distinguished from the typical Aran-Duchenne disease 
which began in the muscles of the thighs. If the disease 
begins in the upper extremities, the legs are, as a rule, not 
affected until very late in the course of the disease ; but 
there are exceptions to this rule. I have observed several 
cases in which the atrophy in the lower extremities began 
almost simultaneously with that in the upper extremities. 

The atrophied muscles in this form exhibit fibrillary con- 
tractions, and, as a rule, present marked changes in electri- 
cal contractility. These changes are not so pronounced as 
in cases of acute or subacute anterior poliomyelitis, and yet 
after the disease has lasted for a considerable length of time 
a typical reaction of degeneration will be found present in 
most of the wasted muscles. In the majority of cases thus 
far recorded the disease has not been distinctly hereditary, 
although series of such cases with distinct hereditary ten- 
dencies have been published by Naunyn, Eichhorst, Ham- 
mond, Osier, and others.* 

* The famous Weathersby family, reported by Hammond, and the Farr family, of 
Vermont, described by Osier, may possibly represent other types of progressive 
muscular atrophy, which we shall consider later on. 



356 THE NERVOUS DISEASES OF CHILDREN. 

As the disease progresses the wasting becomes more and 
more extreme ; the patient is no longer able to get about, 
becomes bedridden, and after many years of annoyance, if 
not of suffering, dies of some intercurrent disease, or from 
extension of the process upward into the region of the me- 
dulla, with consequent paralysis of the vital centres. 

The symptoms of the disease were well described by 
Duchenne, and very little has been added since his day to 
the clinical characterization of this special form. But a very 
warm discussion was waged for a long time regarding the 
origin of the disease, some maintaining its spinal origin, 
others believing it to be a peripheral disease. There was 
some reason for this difference of opinion, for the cases upon 
which the older authors based their views were in part due 
to spinal disease, and in part due to nerve or muscular 
lesions. The microscopical studies of Charcot and Joffroy, 
of Lockhart Clarke, of Hayem, and others, proved beyond 
a doubt that this special form of progressive muscular 
atrophy was due to changes in the spinal cord. The chief 
changes found are these : A sclerotic and pigmentary atro- 
phy of the ganglion cells of the anterior horns, inflamma- 
tory changes in the neuroglia, increased size of the blood- 
vessels, and proliferation of the cellular elements. In fresh 
preparations granular corpuscles are found, and according 
to the degree and stage of the disease the anterior horns 
may be very much reduced in all diameters, and the gan- 
glion cells either atrophied or entirely lost. The anterior 
nerve-roots are affected secondarily to the lesion of the gray 
substance. The nerve-fibres are not all destroyed, a num- 
ber of them remaining intact. Those that are destroyed 
exhibit the appearances of simple atrophy, a point to which 
Charcot alludes as distinguishing these cases from infantile 
spinal paralysis. 

According to these pathological findings we must sup- 
pose that an inflammation spreads slowly from the ganglion 
cells of the anterior horns along the anterior nerve-roots 
without destroying as many of these fibres as is the case in 
infantile poliomyelitis. The atrophic changes in the mus- 
cles are the direct result of irritation, which begins in the 
cells of the anterior horns, and is propagated thence through 



PROGRESSIVE MUSCULAR ATROPHIES. 357 

normal or only half-wasted nerve-roots, to the peripheral 
muscular fibre. Positive as these anatomical findings seem 
to be, it is somewhat surprising to learn how few reliable 
post-mortem examinations have been made in these cases 
proving the correctness of these views. The cases of Pier- 
rot -Troissier, of Strumpell and of Hoffmann, are among 
the few which have been so carefully examined as to have 
placed the spinal origin of this special type of progressive 
muscular atrophy beyond question. In these cases the an- 
terior gray matter was the only part affected, and alone re- 
sponsible for the wide-spread muscular atrophy. 

There is no need in this treatise to give a typical history 
of a case of spinal progressive muscular atrophy, as it oc- 
curs in the adult. I have stated above that these cases are, 
as a rule, not hereditary, and upon this absence of the factor 
of inheritance the differential diagnosis was formerly fre- 
quently based between a progressive amyotrophy and a pro- 
gressive myopathy. But this point of differential diagnosis 
has been rudely shaken by the interesting articles which 
Hoffmann and Werdnig published some years ago concern- 
ing the occurrence of chronic spinal muscular atrophy in 
children. I will endeavor to summarize one of Hoffmann's 
cases of 

HEREDITARY PROGRESSIVE MUSCULAR ATROPHY. 

A girl, four years of age ; the birth of the child was entirely normal ; 
when nine months of age was able to stand ; early abnormal development 
of adipose tissue. Gradually the child became so weak that it could not 
stand, could not sit upright in bed, could not turn around without assistance. 
For a long time it was able to move its feet and its arms. The motor dis- 
turbances increased gradually, and the child lost its superfluous fat and be- 
came thoroughly emaciated, particularly in the trunk and the extremities. 
The face remained full. The sphincters were at no time involved. Mental 
development was good. Speech was normal ; no convulsions ; no strabismus ; 
no difficulties in mastication or deglutition. The child was able to turn its 
head, but could not lift it from the pillow. There was no evidence of any 
hypertrophy or pseudo-hypertrophy in any of the muscles, but there was pa- 
resis and atrophy of the deep muscles of the neck, of the sterno-cleido-mas- 
toid, of the trapezius, of most of the shoulder-muscles, of the latissimus dorsi, 
of the serrati, the pectoral and deltoids, and of the flexor muscles in the arm. 
The biceps and brachialis anticus were very thin, the supinator longus a little 
stronger than these. The triceps was thin and weak. The extensors and 



358 THE NERVOUS DISEASES OF CHILDREN. 

flexors in the forearm were also atrophic and paretic. The thenar and hypo- 
thenar eminences were thin, flaccid, and weak, as were also the individual in- 
terossei. The paralysis was in proportion to the atrophy of these muscles. 
The tendon reflexes were entirely wanting in the upper extremities. The 
mechanical excitability of the muscles was diminished. The nerve-trunks 
were neither thickened nor sensitive on pressure. The paresis and atrophy 
of both upper extremities were entirely symmetrical. There were no fibrillary 
movements. There were no trophic or vasomotor disturbances of the skin 
in the upper extremities. Sensation was entirely normal. There was marked 
diminution of electrical excitability in the median and ulnar nerves, and com- 
plete reaction of degeneration in the biceps. 

The muscles of the back and abdomen were very paretic, the long mus- 
cles of the spine much diminished in volume and power. There was lor- 
dosis of the lumbar region of the spinal column. The gluteal muscles, and 
all the muscles of the thigh, were very atrophic and almost completely para- 
lyzed. The muscles of the leg were also atrophic and paretic. Movements 
of the toes tolerably good. A progressive diminution in the volume of the 
calf and thigh muscles was noted at periods six months apart. No indica- 
tion of any deep reflexes in the lower extremities. The faradic excitability 
of the nerves in the lower extremities was distinctly diminished. No sensory 
disturbances, no fibrillary or fascicular or choreic movements of muscles. 
The paralysis was symmetrical and flaccid. The joints were like those of 
infantile spinal palsy. The child died of an intercurrent pulmonary trouble 
about one year after the first examination. 

The report of the autopsy included the following points : The lumbar 
portion of the spinal cord less in volume than under normal conditions. 
Very marked atrophy of the anterior spinal-cord roots throughout the entire 
spinal cord as high up as the medulla. On microscopical examination the 
chief changes found were in the anterior gray matter and in the anterior 
nerve-roots, from the lower portion of the medulla through the whole spinal 
cord. There was a distinct atrophy and diminution in number of the gan- 
glion cells of the anterior horns throughout the entire spinal cord. This was 
more marked in the lumbar than in the cervical portion. There was also 
very marked atrophy of the anterior roots and a similar affection in the 
peripheral nerves and the nerve-filaments in the muscles, as well as a very 
marked atrophy of the muscles supplied by these nerves. In addition to 
these chief changes there were also slight and symmetrical changes in 
the motor tracts of the spinal cord, particularly in the crossed pyramidal 
tracts and in the lateral columns as well as in the direct pyramidal tracts. 
Medulla oblongata was not involved. 

This case is so similar, from a clinical and anatomical point of view, to 
the typical cases of the Aran-Duchenne type that the close relationship be- 
tween these forms cannot be doubted. It remains to add that an entirely 
similar affection was reported in the case of a brother of the first child, in 
whom the disease began at about the same age and behaved in very much 
the same way. The parents of these two children have raised a family of 
fifteen ; several of these have died of convulsions, others are afflicted with 



PROGRESSIVE MUSCULAR ATROPHIES. 359 

distinct lipomatosis, and the parents invariably accepted the occurrence of 
this excessive accumulation of fat as an evil omen. 

Abortive and hereditary forms of progressive muscular atrophy of the 
spinal type have come under my notice. The cases are those of a physician, 
living in Canada, and his daughter, about twelve years of age ; in both of 
them there was distinct atrophy of the interossei of both hands, flattening of 
the thenar, and slight extension of the atrophy to muscles of the forearm. 
" Weak hands " have been characteristic of the family, but the disease does 
not appear seriously to invade other parts. The father (physician) has had 
this trouble for years, and has been compelled to give up surgical work. 



PROGRESSIVE NEURAL MUSCULAR ATROPHY — PROGRESSIVE 
NEUROTIC MUSCULAR ATROPHY — THE PERONEAL FORM, 
OR LEG TYPE, OF PROGRESSIVE MUSCULAR ATROPHY.* 

In this form of progressive muscular wasting the dis- 
ease begins, in the majority of cases, in the lower extrem- 
ities. At first the extensor muscles of the toes show a 
slight weakness. The small muscles of the feet may be- 
come involved, and then the atrophy spreads very much 
after the fashion of the spreading of the atrophy in the 
spinal cases, from muscle to muscle, until the entire leg is 
considerably atrophied and weakened. As a result of this 
weakening, deformities of the foot may arise ; pes equinus 
or pes equino-varus is a frequent result. In other cases a 
distinct club-foot is developed, and inasmuch as the affec- 
tion may spread quite rapidly from one side to the other, 
a progressive form of wasting in both lower extremities, 
including possibly the development of double club-foot, is 
extremely suggestive of this " leg type " of progressive 
muscular atrophy. The disease, as Hoffmann has shown, in 
rare instances attacks the upper extremities first and then 
involves the lower. Hoffmann has objected to the use of 
the designation " leg type," but since in this form the 
legs are involved at a very early stage, whether the disease 
begins in them or in the upper extremities, it seems proper 
for the present to retain this designation. The atrophy in 
the upper extremities may involve the small muscles of the 
hand, the extensor and flexor muscles of the forearm or the 

* This disease is also known as the type of Charcot-Marie-Tooth. Hoffmann sug- 
gested the term " progressive neurotic," Bernhardt, the term " progressive neural," 
muscular atrophy. 



3 6 ° 



THE NERVOUS DISEASES' OF CHILDREN. 



arm, and may cause a wasting of the muscles about the 
shoulder-girdle. I have seen the infra- and supra-spinati 
especially wasted in several of these cases. The atrophy 
in the upper extremities is, as a rule, not so distinct nor so 
early a symptom as in the cases of the Aran-Duchenne type. 




Fig. 84. — Two Brothers afflicted with the Peroneal Form of Progressive Muscular 
Atrophy, Eight Months and One Year respectively after First Operation. 



Sensory changes are generally present, and serve as an 
important point of differentiation between this special form 
of atrophy and a spinal amyotrophy. The various forms 
of sensation may be slightly altered, or, in some cases, tac- 
tile sensation and temperature sense may remain normal, 
while the pain sense may be more distinctly involved. Par- 
esthesias may be present in addition to the objective 
changes in sensation. The reflexes in the lower extremities 
are either diminished or lost ; the exact state of the reflexes 



PROGRESSIVE MUSCULAR ATROPHIES. 36 1 

depending somewhat upon the stage of the disease at the 
time the patient is examined. The electrical reactions in 
the atrophied muscles are, as a rule, altered. The changes 
are not so extreme as in the cases of spinal amyotrophy, 
nor are they as mild as in the primary muscular dystrophies. 
The reactions are diminished quantitatively, and altered 
also as regards the quality of contractions. A case has not 
yet been reported in which the muscles of the face were 
involved, and there were no changes in sensation, and none 
in electrical reactions in any of the muscles or nerves 
of the neck and head. 

The symptomatology of this rare form will be best elucidated by an 
extract from the account given of the disease as it occurred in two brothers 
which was reported in a paper published by the author in the year 1890. 
These cases are all the more interesting as they are the only ones, to my 
knowledge, which for a time were successfully treated by surgical measures. 

The family history is very meagre. The father, a Bavarian, is dead ; 
cause of death unknown. Mother, living and healthy, thirty-two years of 
age. The two patients were the only children. Both boys were born healthy. 
Each showed disturbances in the use of the legs at a very early day, and at 
the age of five years both had acquired double club-foot. 

When the younger brother was first admitted to the hospital, in 1887, he 
was compelled to use crutches. According to the hospital records there 
was marked shortening of the Achilles tendon and plantar fascia of both feet. 
Foot arched (pes cavus) ; when at rest inner side does not touch the floor. 

Measurements : Right calf, 7 inches ; left calf, 7 inches. Dr. Gibney 
performed double achillotomy. Separation of ends ij£ inch on the left 
and almost the same on the right side. Feet were flexed dorsally to about 
eighty degrees, and plaster-of-Paris splints were supplied. He was dis- 
charged four months after the operation, with a note that the patient walks 
quite well, soles flat on the ground, toes slightly inverted. He returned to 
the hospital in 1888 with paralytic limp and with a position of the feet as rep- 
resented in the accompanying figure. Double achillotomy was again per- 
formed, division of plantar fasciae was made, and there followed application 
of Thomas's tarsoclast and plaster splints with the results as shown in Figs. 
101 and 102. Two months after the operation the feet were in typical cal- 
caneus position, when using his shoes without apparatus ; standing squarely 
on the soles of the feet he shows disposition to roll feet inward. He could 
voluntarily flex the ankle-joint a little beyond ninety degrees, but in so doing 
the toes were hyperextended. He walks very much as children do with a 
peroneal type of poliomyelitis. Marked disposition to pes varus. 

My own examination elicited the following points : The 
boy was of stouter and shorter stature than his brother. 



362 



THE NERVOUS DISEASES OF CHILDREN. 



Intelligence good. His broad chest and fat stomach are in 
curious contrast to his spindle-shaped extremities. Circum- 
ference of chest, 26 inches. Right arm, 6y 2 inches ; left arm, 
1/ inches. Right forearm, 6^ inches; left forearm, 6^ 



inches. Grasp of both hands very weak. A general emaci- 
ation of all parts of upper ex- 
tremities. Very distinct atro- 
phy of infraspinatus. In the 
legs general atrophy is very 
well marked. The right 
thigh, four inches above pa- 
tella, 1 1 inches ; left thigh, 
10*^ inches. Right calf, at 
greatest circumference, 8 
inches ; left calf, 8^ inches. 
The boy walks with a slightly 
waddling gait and has great 
difficulty in climbing the 
stairs. He can raise toes slight- 
ly on the left side, less well 
on the right side. Can raise 
left leg on tiptoe, but cannot 
do this with the right leg. In 
attempting to raise the whole 
body on tiptoe, falls forward. 
Sensation : Tactile sensation 
normal as determined by cot- 
ton, pin test, and the writing 
of numbers on the skin. Tem- 
perature sense normal. Pain 
sense exaggerated. Muscu- 
lar sense normal. Plantar re- 
flexes present and knee-jerk about normal. Slight lividity 
of legs, not so marked, however, as in the case of his 
brother. 




Fig. 85. 
(Same patient as in Fig. 86.) 



The electrical examination : In the upper extremities the faradic response 
in the median and ulnar nerves was decidedly diminished. In the median 
nerve first KCC with 13 MA; ACC not at 20 MA. Galvanic current: In 
the right leg no reactions could be obtained by excitation of the nerves with 
currents used. In the extensor hallucis longus the first KCC and ACC were 



PROGRESSIVE MUSCULAR ATROPHIES. 



363 



obtained with a current of 14 MA. The tibialis anticus did not respond to 
currents of 20 MA. The anterior thigh muscles and posterior thigh muscles 
respond to strong currents of 16 MA, without reversal of formula. In the 
left leg, the extensor hallucis, first KCC with 16 MA ; first ACC with 18 
MA. No contractions could be obtained by direct excitation of the tibialis 
anticus, with currents up to 20 MA ; on excitation of the extensor digitorum 
communis there is a slight movement 
of the small toe. 

The electrical examination, there- 
fore, shows that the reaction of de- 
generation is present in its typical 
form in most of the muscles below 
the patella, the galvanic excitability 
of the peroneal nerve being entirely 
lost. It also shows changes in elec- 
trical behavior in nerves of the upper 
extremities, since the responses of 
the ulnar and median nerves were 
markedly diminished. 

Comparing the histories 
of the two brothers, it was 
noted that they resemble 
each other very closely as 
regards the first appearance 
of the symptoms and the 
manner in which the disease 
spread from muscle to mus- 
cle ; but there were also 
certain differences, such as 
the more marked electrical 
changes in the younger 
brother and the greater in- 
volvement of the upper ex- 
tremities in him, than in the 
older boy. In the younger 
brother the disease was more 
fully developed in every re- 
spect than in his older broth- 
er ; but such variations as occurred were within a reason- 
able limit and will serve to show to what extent variations 
may occur in persons undoubtedly suffering from the same 
type of disease. 




Fig. 86. — The Younger of the Two 
Brothers (see Fig. 84) after Second 
Operation, showing Correction of De- 
formity of Feet, Marked Atrophy of 
Legs, and Incipient Atrophy of Muscles 
above Elbow and around the Shoulder 
Girdle. 



364 THE NERVOUS DISEASES OF CHILDREN. 

In addition to the symptoms which these boys have exhibited, it is inter- 
esting to note that Vizioli has reported the occurrence of amaurosis in a 
similar case, due to an optic-nerve atrophy. This symptom would support 
the argument in favor of the nerve origin of the disease. Furthermore we 
may insist on the fact that hypertrophy has not been recorded in any case, 
and that fibrillary movements seem to occur in some. 

Thomson and Bruce have reported an interesting case of a progressive 
muscular atrophy in a child ; but they nave not attempted to classify it under' 
any special type. The disease began in the lower extremities, and gradually 
extended to the upper, involving both upper extremities and the muscles 
of the back. The gradual changes are well represented in Figs. 87-89. 
The child exhibited some disturbances of sensation (hyperalgesia) ; marked 
paresis in neck, back, and abdomen — most marked in loins, buttocks, and 
legs ; least marked in shoulders and arms ; no hypertrophy of any muscles. 
Electrical reactions at first little altered ; later on faradic excitability consid- 
erably impaired ; more markedly in the legs than in the arms. The report 
of the galvanic responses is too uncertain to permit of positive inferences ; the 
disease was steadily progressive. The case is all the more unique, as the 
authors found a spinal lesion and only very slight changes in the peripheral 



Etiology, — Nothing more need be said upon this head 
than may be inferred from the previous histories. The 
disease is evidently a family affection, sometimes beginning 
at a very early age, as in my own cases, or appearing as 
late as the age of twenty, as in the one case described by 
Charcot-Marie. Whether the cases described by Osier as 
occurring in the Farr family of Vermont, which set in as 
late as the age of forty-six in some of the subjects, belong 
to this category or not is questionable, as the cases were 
reported before this special type of progressive muscular 
atrophy was known. In both my cases a thorough drench- 
ing of the skin by exposure to wet was mentioned, but I can- 
not attribute any further importance to this fact than that 
it may have helped to hasten a disease which was latent in 
the systems of the boys. 

Diagnosis. — The diagnosis of this "leg type," or neural 
form, of progressive muscular atrophy rests upon the recog- 
nition of the early beginning of the disease and of its hered- 
itary or family character. Moreover, the paralysis begin- 
ning in the leg muscles, and spreading to the upper extrem- 

*The case appears to bear a close resemblance to the peroneal form, as well as to 
an hereditary spinal form. 



PROGRESSIVE MUSCULAR ATROPHIES. 



365 




$66 THE NERVOUS DISEASES OF CHILDREN. 

ities is associated with slight changes in sensation in the 
legs as well as in the arms. The occurrence of double 
club-foot (not congenital) will help to make the diagnosis 
still more certain. 

The disease will have to be differentiated from hereditary ataxia, from 
chronic multiple neuritis, from poliomyelitis, and from the primary muscular 
dystrophies. 

From hereditary ataxia the disease can be readily distinguished by the 
absence of the peculiar unsteadiness in walking and standing, by normal 
electrical reactions in cases of hereditary ataxia, and by the persistence of the 
reflexes in many of the cases of the peroneal form. 

From chronic multiple neuritis we can distinguish the " leg type " by the 
fact that pain plays an even greater role in most cases of neuritis ; that the 
atrophy is not steadily progressive, and that neuritis, of however long stand- 
ing, rarely leads to double club-foot ; and furthermore, that neuritis is not apt 
to occur as a family affection. 

From poliomyelitis we can differentiate these cases by the very gradual 
development of the disease, in contrast to the more sudden onset in infantile 
spinal paralysis ; by the progressive development of the atrophy, in contra- 
distinction to the retrogressive character of the wasting in poliomyelitis. 
Poliomyelitis is not an hereditary or family disease, and if the wasting in a 
case of poliomyelitis is as great as in cases of the leg type of progressive 
muscular atrophy the knee-jerks and other deep reflexes will surely be lost, 
while in the leg type they may be preserved for a considerable period of time. 
It is not so easy always to distinguish these cases from the subacute forms of 
poliomyelitis ; but if there is any doubt about the diagnosis in the earlier stages 
of the disease, the further progress of the trouble will remove all uncertainty. 

The disease might also be confounded, in its later stages, with the Aran- 
Duchenne type of progressive muscular atrophy, particularly if, as sometimes 
happens, the atrophy in those cases attacks the legs very soon after it has be- 
gun in the upper extremities. Under such conditions we must rely for the 
differential diagnosis upon the fact that the Aran-Duchenne type begins, as 
a rule, much later in life ; that it is rarely of an hereditary character, and 
that sensation is never affected in those cases as it is in the cases now under 
consideration. But I must concede that the clinical resemblance may be so 
strong between these two forms of disease that it will be practically impossi- 
ble to differentiate between them. 

Pathology. — For a number of years after this disease 
was first described the morbid anatomy was based upon 
mere speculation. Reference was made by several authors, 
among them by Hoffmann, to the older records of post-mor- 
tem examination by Virchow, Friedreich, and others. In 
these cases the authors found a degeneration of the nerves 



PROGRESSIVE MUSCULAR ATROPHIES. 367 

and a degeneration of the columns of Goll, but no satis- 
factory statement could be made at that time, which ante- 
dated the discovery of modern staining methods, regarding 
the disease of the anterior ganglion cells. I objected to the 
use of these older records as a proof of the non-involvement 
of the spinal cord, but all doubt regarding the origin of at 
least some of these cases has been removed by the studies 
of Marinesco, of Dubreuilh (Revue de Medecine, 1890, p. 441) 
in a very typical case of a child dying, in a family of which 
the mother and eleven children were affected with the same 
trouble. Dubreuilh proves that there were old changes in 
the peripheral nerves, particularly in the motor nerves of the 
hands and feet, and that these changes diminished toward 
the spinal cord. The gray substance of the spinal cord 
was normal; there was a slight increase of the glia in the 
column of Goll, but not a true sclerosis, and the nerve-fibres 
were not diminished. The changes in the muscles consisted 
of simple atrophy of the fibres, of a loss of transverse stri- 
ation, and of a proliferation of the nuclei. There were also 
some degenerated fibres and some in a condition of hyper- 
trophy. It will be seen from this post-mortem account that 
the changes in the spinal cord were evidently secondary to 
those in the peripheral nerves ; furthermore, that the changes 
in the muscles resembled more closely those occurring in 
the primary dystrophies than in diseases due to spinal proc- 
esses. This form evidently holds a median position between 
the true spinal amyotrophies and the primary myopathies. 
(Cohn, Cassirer, and Oppenheim hold this same view.) The 
purely neural origin of the disease is in doubt. Later in- 
vestigations may lend some coloring to the proposition of 
Bernhardt to speak of the cases as spinal neuritic atrophies, 
since the ganglion cells of the anterior horns, the anterior 
nerve-roots, and the peripheral nerves, after all, constitute 
a physiological unit. Further post-mortem examinations of 
the subjects of this disease may exhibit more considerable 
changes in the spinal cord than those which were found in 
Dubreuilh's case. I cannot consider the question settled, 
in spite of the autopsies recorded by Siemerling, Sainton, 
and others, and would urge a careful and detailed examina- 
tion of every such case if opportunity presents itself. 



368 THE NERVOUS DISEASES OF CHILDREN. 

The general treatment of these cases will be referred to 
in connection with the discussion of the other forms of mus- 
cular disturbance. I only wish to insist once more on 
the fact that the orthopedic surgeon may in these cases pro- 
cure moderate relief, at least for a considerable period of 
time, by such measures as were employed by Dr. Gibney 
in the cases of the two boys under my immediate care. 

PRIMARY MYOPATHIES (PRIMARY MUSCULAR DYSTROPHIES). 

Our knowledge of these dystrophies is a recent acquisi- 
tion for which we are specially indebted to the brilliant 
clinical and pathological studies of Erb. The German 
neurologist not only described a new form of progressive 
myopathy, but demonstrated very clearly the relations of 
this form to the older and well-known form of muscular 
pseudo-hypertrophy. In addition to this, Erb has sub- 
jected the innumerable types of muscular atrophy to a 
healthful criticism, and has thus helped to correct many er- 
roneous views which were advanced by others. Excellent 
work has also been done by Charcot, Landouzy, Dejerine, 
Schultze, Striimpell, Dercum, Spiller, and others. 

For a long time the Aran-Duchenne type of progressive muscular atrophy 
and muscular pseudo-hypertrophy were the only well-known forms of pro- 
gressive muscular disease. With the evidence which proved that pseudo-hy- 
pertrophy was never of spinal origin, a wide distinction was created between 
the spinal forms and the primary myopathies. As new types of muscular 
diseases were described by individual authors the question arose in each in- 
stance, whether the cases reported inclined rather to the spinal than to the 
pseudo-hypertrophic form. It is only very recently that sufficient evidence 
has been brought forth to show that all the primary myopathies are closely 
related to one another, and that the types that have been so carefully de- 
scribed in former years, are practically nothing but peculiarities in the topo- 
graphical distribution of diseases which should be included under the broad 
term of progressive muscular dystrophies. Much was at one time made of 
the occurrence of atrophy or hypertrophy, but less importance is attached to 
this point now, for we know that the hypertrophic stage represents in most 
instances an earlier stage of the disease, and that atrophy rapidly supervenes 
upon this apparent or real hypertrophy of muscular tissue. Hypertrophy 
never occurs in the spinal forms of muscular wasting,* and in this respect it 

* Single hypertrophied fibres have been found in sections of muscles from cases of 
progressive amyotrophy. 



PROGRESSIVE MUSCULAR ATROPHIES. 



369 



is a most significant symptom, but its occurrence is not a sufficient basis for 
a classification of the various types of primary myopathy. 

Before entering upon a detailed account of the primary 
myopathies, it will be well to state the chief features of 
the various types. By placing the symptoms in parallel 
columns the entire subject can be understood more readily : 

Types of Primary Dystrophies. 



Part first affect- 
ed. 

Distribution of 
hypertrophy. 

D i s t r i b u tion 
of atrophy. 



Parts remain- 
ing normal. 



Muscular Pseudo-hy- 
pertrophy. 



Legs (calves). 



Calves, rarely thighs. 



Thighs, deep muscles 
of back, shoulder, and 
scapular muscles. 
Calves during later 
period ; at that time 
also general atrophy. 

Face, forearm, and 
hand, except in last 
stages. 



Juvenile form of Pro- 
gressive MUSCULAR 

ATROPHY (Erb'S TYPE). 



Shoulder girdle. 

Muscles around shoul- 
der-girdle and pelvic 
girdle. 

Thighs, deep muscles 
of back, upper arm. 
Hypertrophied parts 
may become atrophic 
in later stage. 

Face, forearm, hand 
and leg muscles, ex- 
cept in last stages. 



Type Landouzy- 

Dejerine. 

("Infantile Form.") 



Face and shoulder- 
girdle. 

None. 



Face muscles, includ- 
ing lips and orbicu- 
lar i s palpebrarum ; 
shoulder and scapu- 
lar muscles. 

Forearm, hand and 
legs, and deep mus- 
cles of back. 



If we add to the above table the facts that in all these 
three forms there is a distinct history of heredity, or at least 
of the occurrence in various members of the same family; 
that there are often slight and sometimes very marked quan- 
titative changes in the electrical reactions, but that there is 
rarely if ever a complete reaction of degeneration ; if we 
note, furthermore, that the reflexes may remain preserved 
for a considerable period of time and then disappear, in 
keeping with the progress of the muscular wasting, we 
shall see that there is practically no other distinction be- 
tween these various types of myopathies than the mere 
distribution of atrophy or hypertrophy. The hereditary 
type of progressive muscular atrophy as it was described 
by Leyden, is not included, simply because heredity is not 
a sufficient basis of classification, and many of his cases 
would belong more properly either under the heading of 
Erb's type, or under that of the peroneal form of progres- 



370 



THE NERVOUS DISEASES OF CHILDREN. 



sive muscular atrophy. There appears at first sight to be 
a broad gap between the cases of muscular pseudo-hyper- 
trophy and the cases of Erb's type, but these different types 
may be represented in various members of a single family. 
The relation of these types to one another is convincingly 
demonstrated by the cases of three brothers to be referred 

to in this chapter. (Figs. 90- 
92.) 

Landouzy - Dejerine have 
insisted on the right of their 
type to special consideration, 
claiming that ordinary muscu- 
lar pseudo-hypertrophy, and 
even Erb's type of disease, 
were never associated with 
an involvement of the face, 
yet it is very certain that their 
type is practically nothing 
more than that described by 
Erb, plus involvement of the 
face muscles. Landouzy-De- 
jerine also denied that an at- 
rophy of the face muscles 
was ever associated with typ- 
ical pseudo-hypertrophy ; but 
Westphal first published a 
case which showed that the 
face muscles are occasionally 
affected in cases of typical 
pseudo -hypertrophy, and I 
was able, some years ago, to 
record a case which was a 
typical representative of mus- 
cular pseudo- hypertrophy, 
which passed through the 
stage defined by Erb's type, 
and in which the muscles of 
the face were also involved. There is, therefore, no suffi- 
cient reason to retain the Landouzy-Dejerine type as a sep* 
arate form of disease. 




Fig. 90. — Oldest Brother, aged Sixteen 
Years, exhibiting Atrophy following 
Pseudo-Hypertrophy of the Calves, 
and Extreme Atrophy of Shoulder 
Muscles. Boy unable to move from 
chair or to hold himself erect (late 
stage of "pseudo-hypertrophy"). Figs. 
106-108 represent three brothers. 



PROGRESSIVE MUSCULAR ATROPHIES. 



3/1 



The characteristic symptoms of the various types may 
now be described. 

Muscular Pseudo-hypertrophy.— As described many 
years ago, above all by Meryon, Duchenne, and Gowers, 
pseudo-muscular hypertrophy is characterized by its oc- 
currence in early youth. 
Boys are affected some- 
what more frequently 
than girls ; but although 
affecting boys, the dis- 
ease is inherited almost 
invariably through the 
mother. The first symp- 
toms are, a weakness in 
the muscles of the leg 
and an early increase in 
the size of the calf mus- 
cles. In rare instances 
the hypertrophy may be- 
gin in the thigh muscles. 
The gait is waddling, and 
the child soon finds diffi- 
culty in walking up and 
down stairs, in climbing 
on chairs, in rising from 
the floor or from any re- 
cumbent posture. In the 
earlier stages of the dis- 
ease the patient rises from 
the floor by dint of great 
effort (see Fig. 109) and 
by " climbing up upon 
himself." In later stages 
of the disease the patient, 
if put on the floor, lies 
absolutely prostrate and 
is not even able to raise 
the head from the floor. 

Sitting up without support may be entirelv impossible. As 
the weakness and atrophy increase, the patient becomes more 




Fig. 91. — Second Brother, aged Thirteen and 
a Half Years, exhibiting Hypertrophy of 
Calves, of Gluteal Muscles, and of Muscles 
about the Shoulder Girdle ; Distinct Atrophy 
of Arm Muscles (Erb's Type, or Juvenile 
Form of Progressive Muscular Atrophy). 
All the muscles are now beginning to atro- 
phy ; boy is only a little less helpless than his 
older brother. 



372 



THE NERVOUS DISEASES OF CHILDREN. 



and more helpless, is unable to stand or to walk, becomes 
either bedridden, or is compelled to sit in a chair and even 
loses the use of the upper extremities ; is not able to raise 




Fig. 92. — Youngest of the Three Brothers, in the Earlier Stage of Pseudo-Hypertrophy. 
(For the photographs of this boy I am indebted to the courtesy of Dr. Collins.) 



the arms, and may have no use of any of the muscles, except 
the small muscles of the hand. In the two boys (Figs. 90, 
91) under my observation, a climbing up of the hand along 



PROGRESSIVE MUSCULAR ATROPHIES. 



373 



the head in order to get the arm into the erect position was 
a very characteristic feature. 

In addition to the hypertrophy of the calf muscles, we 
now know that there is apt to be atrophy of the muscles of 
the thigh, of the arm, and the shoulders ; the scapular raus- 




FiG. 93. — Boy with Pseudo-Hypertrophy attempting to straighten himself. Same 

patient as in Fig. 92. 



cles, and at a very early stage of the disease the serrati, the 
latissimus dorsi, and the pectoralis major, are often wasted. 
The forearm muscles and the hand muscles are rarely af- 
fected. The disease is often associated, as the other forms 
of myopathy may be, with symptoms of a general degener- 
ation ; thus I have found nystagmus, lisping speech, and a 
moderate degree of imbecility in not a few of these cases. 



374 



THE NERVOUS DISEASES OF CHILDREN. 



Erb's Type, or the Juvenile Form of Progressive 
Muscular Atrophy. — According to Erb's own summary, 
this type is characterized by progressive wasting with 
weakness of certain groups of muscles, beginning either in 
childhood or early youth, involving, as a rule, the muscles 
of the shoulder girdle, the upper arm, the pelvic girdle, the 
thigh, and the back ; the forearm and leg muscles remain- 
ing intact for a very long time. The atrophy may be asso- 
ciated with true or pseudo-hypertrophy of some muscles. 

The pectorals, trapezii, la- 
tissimi dorsi, the serrati, the 
rhomboids, as well as most 
of the upper-arm muscles 
and supinators are apt to 
be wasted ; while the del- 
toids, supraspinal, and in- 
fraspinati are either normal 
for a long time or hyper- 
trophied. There are no 
fibrillary contractions and 
no reaction of degenera- 
tion ; no sensory or visce- 
ral disturbances. (See Fig. 

9 i.) 

A few years ago, I was 
inclined to consider Erb's 
type a great rarity in this 
country; it is unquestion- 
ably the least frequent of 
all the forms of progressive muscular atrophy which we 
have occasion to see in clinics or private practice ; but I 
have seen at least a dozen cases of this form within a period 
of five years, from which the reader may gather the fre- 
quency or infrequency of the disease; but there is no doubt 
that many cases exist which have not been reported, simply 
because they have not been properly recognized. 

The Facio-Scapulo-Humeral, or Landouzy-Deje- 
rine Type. — This type includes cases in which the atrophy 
begins early in life, and, as a rule, in the muscles of the face, 
giving rise to what the authors have termed the " fades my~ 




Fig. 94. — Patient with Landouzy-Dejerine 
Type. Indication of bouche de tapir; 
patient cannot show upper teeth, nor 
close eyes. (See also Fig. 8.) 



PROGRESSIVE MUSCULAR ATROPHIES. 375 

opathique /" the lips are considerably thickened and consti- 
tute the bouche de tapir, or tapir mouth. Later on in the 
course of the disease the atrophy spreads to the shoulder 
and arm muscles. The supraspinati and infraspinati, the 
subscapularis, and flexors of the hands and lingers remain 
normal. Among these muscles that remain normal it may 
at once be noted are several which are distinctly hypertro- 
phied in Erb's type. In the Landouzy-Dejerine type the 
muscles of deglutition, of mastication, the respiratory and 
laryngeal muscles, as well as the ocular muscles, remain nor- 
mal. In exceptional cases the disease may begin in the 
shoulder or arm muscles, or even in the lower extremities. 
The disease is distinctly hereditary. Fibrillary contrac- 
tions and reaction of degeneration are never present. 

I have given these symptoms as nearly as possible as 
they were stated by the authors themselves in order to do 
full justice to their cause, but it will be evident, without 
further argument, that, with the exception of the involve- 
ment of the face, there is very little distinction between 
these cases and those of the juvenile form of progressive 
muscular atrophy. 

The various types of progressive myopathy are suffi- 
ciently illustrated by Figs. 90-94, which supply additional 
evidence in favor of the intimate kinship existing between 
these various types. 

Figs. 90 and 91 represent two brothers who had been under my obser- 
vation for many years ; the one is an example of muscular pseudo-hyper- 
trophy with atrophy of shoulder and trunk muscles ; the other boy's disease 
began as a muscular pseudo-hypertrophy, but the hypertrophy of the muscles 
above the shoulder and pelvic girdles constitute it a most pronounced case of 
Erb's type — the juvenile form of progressive muscular atrophy. A third 
brother was seen in the state of pseudo-hypertrophy (Fig. 92). After a lapse 
of years the pseudo-hypertrophy may disappear, and all three brothers will 
then bear the closest resemblance to each other. Fig. 94 is a photo- 
graph of the patient afflicted with the Landouzy-Dejerine type of muscular 
atrophy. 

To these I wish to add a brief history (published in 1890), of a case 
which was one of unusual importance, as the patient presented the combined 
symptoms of all known types of primary myopathy. A young man, twenty 
years of age, whose history is entirely negative, has noted, since early childhood, 
a peculiarity about his face ; he was not able to whistle as well as other boys, 



376 THE NERVOUS DISEASES OF CHILDREN. 

and as long as he can remember his face appeared twisted to one side, at 
least while speaking. It was not until one year before my examination that 
he became aware of any further trouble. He claims to have struck his 
shoulder, and since that time to have noticed a weakness of both upper ex- 
tremities. He was employed by a surgical-instrument maker and had to lift 
heavy boxes, but this he is no longer able to do. 

During the examination, was asked to whistle ; he could not do it, nor 
could he keep his eyelids tightly pinched. The condition of his muscles may 
be summarized as follows : 

Wasted: Both pectorals, major and minor. Both serrati, right more 
than left. Both latissimi dorsi, left more than right. Levatores anguli 
scapulas, right more than left. Rhomboids, left more than right. Both tra- 
pezii (middle and lower third), left more than right. Both biceps muscles 
and both brachiales-antici. Both triceps muscles, right more than left ; the 
supinator longus of both arms, right more than left. Anterior thigh and leg 
muscles, left more than right. Posterior thigh muscles (thin). Orbicularis 
palpebrarum of each side and the orbicularis oris. 

Normal : Back muscles, forearm and hand muscles, gluteal muscles, and 
muscles of foot. 

Hypertrophied : Deltoids, infraspinati, supraspinati, and calf muscles, but 
the right calf is beginning to waste. 

There were no fibrillary contractions in any of the affected muscles. All 
the muscles, including those wasted and those hypertrophied, responded to 
both currents in proportion to the quantity of normal contractile fibre that 
each muscle retained. There was no reaction of degeneration in any muscle. 
The knee-jerks were present. There was no ataxia, no disturbance of sen- 
sation, and there was not a single symptom pointing to an involvement of the 
central nervous system. 

From the condition of the calves, there might be some reason to class this 
case among the pseudo-hypertrophies ; from the appearance of the shoulder- 
girdle and the thinness of the upper arm, we might rank it with Erb's juve- 
nile form ; and if we take the face into consideration, we might classify it 
with the Landouzy-Dejerine type of progressive muscular atrophy. It does 
not quite tally with the older accounts of pseudo-hypertrophy ; for although 
the shoulder muscles are sometimes involved in such cases, it is exceptional 
to have both shoulder and face muscles affected. From Erb's form it is dis- 
tinguished by the involvement of the face muscles, and from the ordinary 
cases of the Landouzy-Dejerine type this case is to be distinguished by the 
additional involvement of the calves. It will not do to suppose that the boy 
is affected with three different diseases ; it is much more to the point to state 
that the symptoms in this case prove that the three distinct forms practically 
represent subdivisions of one and the same disease, and that the primary 
muscular disease was so fully developed in this patient that he practically 
represented all the known types of progressive myopathy. We see by this 
case, too, how wrong it is to make too much ado over the varying distribu- 
tion of atrophies or hypertrophies. 



PROGRESSIVE MUSCULAR ATROPHIES. ^77 

Diagnosis. — It is much more difficult to differentiate 
between the various forms of progressive muscular dys- 
trophies than to distinguish between them and other dis- 
eases. The factor of heredity, the occurrence in families, 
the absence of fibrillary twitchings and of changes in elec- 
trical reaction, and above all the slowly progressive char- 
acter of these diseases and their onset in early life, will 
scarcely permit of any confusion with other diseases. If 
the patients are examined later in life, after extensive atro- 
phy has supervened upon preceding hypertrophy, if the 
atrophic paralysis is so extreme that the patient is bedrid- 
den and that there are practically no muscles which re- 
spond to the electrical current, if all the reflexes are absent, 
there may be considerable difficulty in differentiating be- 
tween such a condition and that of chronic poliomyelitis. 
But even at such an advanced period of the disease the 
former history of the patient will help to establish a correct 
diagnosis. 



Mention should, however, be made of one other condition which I have 
met with but twice, in which the question has come up whether the patient 
was suffering from a form of primary muscular dystrophy. Both of these 
cases were instances of what I would wish to call physiological hypertrophy. 
The one was the case of a physician who had attained unusual muscular 
development in his efforts to correct phthisical tendencies, and the second 
was the case of a brother of a well-known physician who, through inordi- 
nate exercising at lawn tennis had produced a hypertrophy of the shoulder, 
arm, and forearm muscles of the right side. In both these cases I was con- 
sulted because of a weakness which had followed upon this unusual hyper- 
trophy. In the case of the physician first referred to, several muscles (among 
them the infra and supra spinati, and the deltoid) had begun to atrophy dis- 
tinctly. The possibility of the development of some form of progressive 
muscular atrophy was entertained by others in both cases, but I was certain 
that this grave prognosis was not justified, for the entire development of the 
trouble, the onset late in life, and the occurrence of these conditions in per- 
sons with a clear family record furnished the best evidence that the condition 
of these two patients was the result of over-exercise and nothing more. 

The point of greatest interest in both these cases is the surprising weak- 
ness of the muscles in spite of the hypertrophied condition. It would seem 
that any muscular fibre forced to an unnatural growth (hypertrophy) is 
likely to succumb in the struggle. In both these cases I insisted on absolute 
rest of the affected parts and on the use of common sense in physical exer- 
cise. In the case of the physician all the muscles have returned to a normal 



378 THE NERVOUS DISEASES OF CHILDREN. 

state and have remained so for fully three years ; in the second case the 
normal strength of the arm has returned, and there is no sign of an impend- 
ing atrophy. 

The points of differential diagnosis between the spinal 
forms of progressive muscular atrophy and the primary 
dystrophies have been insisted upon over and over again. 
The rule is that in spinal cases the affection is not hered- 
itary and generally begins in the upper extremity ; there 
are fibrillary contractions, and marked changes in electri- 
cal reaction ; while in the dystrophies heredity is the most 
prominent factor, the diseases begin early in life, there are 
no fibrillary contractions, and the electrical reactions re- 
main normal or nearly so throughout the entire course of 
the disease until the stage of extreme atrophy is reached. 

After the consideration of these cardinal symptoms there should be no 
real difficulty in distinguishing between these two principal forms of progres- 
sive muscular wasting ; yet cases appear every now and then in which the 
symptoms are so distributed that it is impossible to classify them by adhering 
to these cardinal symptoms. The most interesting case in point is unquestion- 
ably the one reported by Striimpell. In this patient the disease did not begin 
until the age of twenty-nine, in the fingers of the right hand, but there was a 
strong history of heredity, his mother having suffered from a similar disease. 
The atrophy spread from the small muscles of the right hand to the muscles 
of the shoulder, and later on to the muscles of the opposite arm. The deep 
muscles of the spine, the glutei, and the thigh muscles remained entirely in- 
tact, after a period of more than eleven years. The symptoms just stated 
pointed to a spinal form, rather than to a pure myopathy ; the heredity 
was, however, more in keeping with the primary dystrophies, and the entire 
absence of fibrillary movements as well as of changes in the electrical re- 
actions would have inclined one to place the case in the category of primary 
myopathies. The histological character of the muscular tissue was also more 
like that described in cases of primary myopathies, yet at the autopsy de- 
cided changes were found in the spinal cord, in the anterior nerve-roots, and 
in a number of the peripheral nerves. Thus the impropriety of classing such 
a case exclusively under one heading or the other was clearly demonstrated, 
and I think Striimpell quite right in insisting that the chief value of his case 
was in showing that these cases of progressive muscular atrophy, whatever 
form they take, belong to the order of hereditary systemic diseases, and that 
it is largely a matter of chance or else due to causes still unknown whether 
the peripheral or the more central portion of the second division of the mo- 
tor tract becomes the chief seat of the disease. A case of Savill's' shows 
that the symptoms of an amyotrophy may coexist with those of a myopathy 
of the Landouzy-Dejerine type. 



PROGRESSIVE MUSCULAR ATROPHIES. 379 

PATHOLOGY. — The designations, " primary myopathy," 
or " primary muscular dystrophies," which have now been 
universally accepted for the diseases under consideration, are 
meant to imply that the origin of the disease is in the mus- 
cular system itself, and that it is not due to changes either 
in the peripheral nerves or in the spinal cord. With regard 
to the more recently described forms of primary muscular 
dystrophy, there has never been any doubt as to the non- 
spinal origin, but a long and hot discussion was waged over 
the spinal origin of muscular pseudo-hypertrophy. In 1888 I 
analyzed all the cases of muscular pseudo-hypertrophy that 
were accessible at the time, and found that of twenty-five 
cases which had been reported, eight had to be excluded 
because the spinal cord was not examined microscopically, 
or because the examination was not properly made. Of 
the seventeen remaining cases, the spinal cord and anterior 
nerve-roots were found absolutely normal in twelve, and in 
five others the changes that were found could not be held 
responsible for the changes in the muscles. A similar con- 
clusion has been reached by other authors, and at the pres- 
ent time no author of repute has ventured to fall back 
upon the older theory of the spinal origin of muscular 
pseudo-hypertrophy. Such slight changes as were observed 
by some — the diminution, for instance, in the number of 
ganglion cells or slight changes in the contours of these 
cells — can now be sufficiently explained on the supposition 
that all such changes are secondary to the peripheral mus- 
cular trouble. 

The histological condition of the atrophied or hypertro- 
phied muscles has been studied with particular care, for it 
was supposed for a very long time that the diagnosis of a myo- 
pathy or of an amyotrophy could be based safely enough 
upon histological appearances ; but we shall see that this 
hope of finding some absolute point of differentiation be- 
tween these two conditions can no longer be entertained. 
In muscles atrophied from spinal lesions, the following were 
supposed to be the chief changes : a loss of striation of the 
muscular fibres and a narrowing of the same, an increase 
in the number of muscle nuclei, and possibly segmentation 
of the nuclei, granular or fatty degeneration of the fibres, 



380 THE NERVOUS DISEASES OF CHILDREN. 

and occasionally globules of fat between the muscle fibrils. 
Some or all of these changes are present according to the 
length of time that the atrophy has existed, but all of these 
changes have also been found in spinal forms, as well as 
in primary dystrophies. The increase in the nuclei is not 
so great in the dystrophies as in the amyotrophies. In the 
spinal forms it was thought that hypertrophied fibres were 
never found, while they are extremely common in the 
purely muscular types. But Mueller and' others have re- 
corded exceptions to this rule and have proved the presence 
of hypertrophied fibres even in cases of poliomyelitis of 
old standing. A large number of hypertrophied fibres in 
a given section of a muscle is, nevertheless, more frequent 
in the primary muscular dystrophies than in the spinal 
atrophies. 

The next question that arose was whether it was possible by histological 
examination to differentiate between the various primary myopathies. In 
muscular pseudo- hypertrophy we have, as a rule, a narrowing of the fibres 
with changes in their contour, granular or fatty degeneration of the fibrils, 
and accumulation of fat globules between them, and increase, without marked 
proliferation, of the connective tissue. Hypertrophied fibres can be found 
scattered in between fibres of normal dimensions, or between those that have 
evidently undergone atrophy. A slight increase in the muscle nuclei is often 
found, but unusual increase is suspicious of a spinal origin. Jacoby thought 
that the disease consisted in the main of a chronic inflammation invading 
both the perimysium and the muscle tissue, and was inclined to term the 
process a myositis progressiva hyperplastica, but his views and his histo- 
logical findings have not been corroborated by others. Westphal, in a very 
typical case of pseudo-hypertrophy, found, on post-mortem examination, 
enormous increase of adipose tissue in which the muscle fibrils were nearly 
of normal size, increase of the interstitial connective tissue, no hyper- 
trophied fibres, and strands of connective tissue occasionally passing 
through the fatty parts ; a few of the groups of muscle fibres appearing 
to be strangulated by the strands of connective tissue. In sections from 
a case which I have recently had occasion to examine, the fibres were found 
to be of varying size and there was a distinct proliferation of the muscle 
nuclei. Schultze, in a case which stands midway between pseudo-hyper- 
trophy and Erb's juvenile form, found, in addition to peculiar giant-cell for- 
mations, a large number of fat-cells in the muscular tissue, an increase of the 
connective tissue, and remnants of hypertrophied, normal, and atrophic fibres, 
and an enormous increase of nuclei, which the author thought greater than 
in the ordinary cases of pseudo-hypertrophy. He also described the occur- 
rence of vacuoles which were in all probability not due to the hardening 



PROGRESSIVE MUSCULAR ATROPHIES. 



381 




Fig. 95. — Changes in Muscular Tissue in a Case 
of Primary Dystrophy. (Erb.) a, Above altered 
blood-vessel ; for other lettering see text. 



process. Hitzig's observations were of special interest for a time, as he 
examined four cases most carefully. He concluded that " the primary and 
most important change in juvenile atrophy is not an interstitial process, but 
decidedly parenchymatous, and according to the intensity of the disease is 
represented by slight or ex- a 

cessive hypertrophy of the 
fibres . . . The anatom- 
ical changes in pseudo-hyper- 
trophy, on the other hand, 
are characterized by active 
changes in the connective 
tissue." This distinction be- 
tween the histological changes 
in pseudo - hypertrophy and 
Erb's form of primary myop- 
athy has not been borne out 
by others, and if I am not 
mistaken, Hitzig has with- 
drawn his former views. The matter was definitely settled by more recent 
studies of Erb, Cramer, Pick, and others (Figs. 95 and 96). Erb proved that 
in all cases of progressive muscular dystrophies, whether of one type or 
another, the changes in the muscular tissue were very similar, and that 
such changes as did occur were simply due to different stages of the dis- 
ease. Erb showed (Fig. 95) that hypertrophy and atrophy of fibres, marked 
proliferation of the nuclei, vacuolization (near b), and segmentation (c) of 
fibres were the chief changes, and that these occurred in all possible forms of 
primary dystrophies, and of these phenomena the hypertrophy of the fibres 

seemed to be the first to ap- 
pear. 

From a case of the Lan- 
douzy- Dejerine type (Fig. 
97), I excised a large piece 
of the infra-spinatus muscle, 
and in doing this a piece of 
the nerve as it enters the 
muscle was accidently re- 
moved. On histological ex- 
amination of the specimen, 
prepared for me by Dr. 
Wiener, we found a consid- 
erable amount of fatty tissue between and around the muscular fibres (Figs. 
97, 98) much atrophy, but no hypertrophy of the muscle fibres; there was 
also distinct evidence of a marked degeneration of the nerves. The finding 
was a very singular one, but it will not do to draw too broad an inference 
from a single case. At all events, the participation of the nerve (whether in 
primary or secondary fashion) in a disease supposed to be a purely muscular 
affection is worthy of note. 




Fig. 96. 

Fibres 



AN. b 

-a, Normal Fibres ; b, Hypertrophied 
c, Atrophied Fibres and Fat. (Erb.) 



382 



THE NERVOUS DISEASES OF CHILDREN. 



After a very long discussion on the histological changes 
in progressive muscular dystrophies, Erb concludes that 












^i^'Sr^BPp* 



Fig. 97. — Section of Infra-Spinatus Muscle from a Case of Landouzy-Dejerine Type, 
showing Deposits of Fat and Degenerated Bundles of Nerves. (Low power. Draw- 
ing made after specimen stained according to Van Gieson.) 

these dystrophies are forms of a tropho- neurosis, which 
may occasionally be the result of functional disturbance of 
trophic centres ; and that such disturbance may be either 



A 



Fig. 98. — A Part of the Nerve Bundles more highly magnified, showing Degenera- 
tion of Nerve Fibres. (See preceding figure.) 

primary or secondary to spinal lesions — a conclusion not 
unlike that reached by Strumpell a few years later in the 
article referred to above. 



PROGRESSIVE MUSCULAR ATROPHIES. 



383 



Duration. — All these forms of muscular dystrophies take 
a relatively slow course, but the period of time that elapses 
before the stage of utter helplessness is reached varies very 
much in different individuals. It is safe to say that those 
suffering from muscular pseudo-hypertrophy are the ones 
most apt to be completely crippled at an early day. Two 
or three years often suffice to make the patient entirely help- 
less. Erb's juvenile form 
and the Landouzy-Dejerine 
type progress more slowly 
— a number of years elaps- 
ing before the legs become 
involved. I have seen one 
case of Erb's form which 
has lasted over thirty years, 
and another of the typical 
muscular pseudo-hypertro- 
phy, which has lasted at 
least fifteen ; but if death 
does not ensue from the 
disease itself, patients may 
be carried off by any slight 
intercurrent disease. 

Treatment. — Interest- 
ing as these diseases are 
from a medical and scien- 
tific point of view, they are 
unfortunately most unsat- 
isfactory as regards the re- 
sults of treatment ; and yet 
there is no need of despairing and declaring that nothing 
can be done in the earlier stages of these troubles. Con- 
tractures of muscles and deformities of various parts are 
extremely annoying and prevent the patient from getting 
about freely, or without the use of crutches or wheeling- 
chairs. I wish to plead for the early and active interfer- 
ence of the orthopedic surgeon. As the diseases are at 
times but very slowly progressive, the correction of de- 
formities by surgical measures may bring relief for a con- 
siderable period of time. Excellent though temporary 




Fig. 99. — Boy with Defective Development 
of Scapula and Shoulder Muscles. 



384 



THE NERVOUS DISEASES OF CHILDREN. 



results were obtained in several patients whom I have 
treated in conjunction with Dr. Gibney. His results in the 
cases of the peroneal form of progressive muscular atrophy 
make one a little hopeful that still better results may be 
attained in the future in this and other forms of muscular 
atrophy by similar or even bolder measures ; but as Op- 
penheim has recently insisted, such cases are not suitable 

for tendon transplantation. 
Intelligent exercise of 
atrophied or hypertro- 
phied muscles, diligent 
and prudent use of mas- 
sage and electricity in these 
cases will be in order. The 
latter agent has a whole- 
some influence upon the 
condition of muscles whose 
nutrition has been altered, 
and is furthermore a con- 
venient form of moderate 
exercise. In the case of a 
boy suffering from the 
Landouzy - Dejerine type, 
all the symptoms were 
brought to a standstill, and 
a decided improvement 
has been effected by the 
careful administration of 
electricity. If the stage of 
atrophy and helplessness 
has been reached, nothing more can be done than to make 
the patient as comfortable as possible. 




Fig. ioo. 



TOTAL ABSENCE AND EARLY ATROPHY OF MUSCLES. 

This condition is suggested by the preceding discussion 
of the primary muscular dystrophies, and may as well be 
referred to in this connection as in any other. The condi- 
tion is a rare one. The following case, which I have seen, 
suggests a similar condition, and at the same time shows 



PROGRESSIVE MUSCULAR ATROPHIES. 38$ 

that the defect may include bony parts as well as the mus- 
cles covering them. 

The boy whose condition is represented in Figs. 99 and 
100 was seen by me a few years ago. He was then six 
years of age. The mother stated that she had noticed dur- 
ing the past year that the right shoulder was higher than 
the left. On examination we found that there was shorten- 
ing of the right scapula, the longest diameter being 4 ctm. 
less than that of the left, and there was insufficient develop- 
ment of the right supra- and infra-spinati of the latissimus 
dorsi, and of the pectoralis major. It will be noted that 
these are the very muscles which are often involved in 
cases of primary muscular dystrophies. The condition is 
of some interest as showing that the muscles which are most 
subject to disease are also found congenitally defective. 
Whether this defect of development is due to some periph- 
eral nerve anomaly, or whether it is the result of defective 
development of the spinal-cord cells, cannot be stated. 

Dejerine and Thomas have described a form of disease 
which they designate as an " hypertrophic and progressive 
interstitial neuritis of childhood." It bears some resem- 
blance to the peroneal form of progressive muscular atrophy, 
and to hereditary ataxia. The chief symptoms are: Progres- 
sive wasting of the muscles at the distal ends of the extremi- 
ties, lightning pains, Romberg symptom, contracted pupils, 
sluggish reaction of pupils or complete immobility, nystag- 
mus, diminution of electrical responses, hardening of nerve 
trunks as determined by palpation. Anatomical findings 
were : Chronic interstitial neuritis, most marked at the 
periphery ; sclerosis of the columns of Goll and of Burdach ; 
atrophy of the anterior horns and anterior roots. 



CHAPTER XXII. 

MALFORMATIONS AND CONDITIONS DUE TO DEFECTIVE 
DEVELOPMENT OF THE CORD. 

Arrest of, or disturbance in, the development of the 
spinal cord is generally associated with similar conditions 
affecting the brain. These spinal-cord conditions have, with 
the exception of spina bifida, very little clinical interest. 
The chief malformations are the following : 

Amyelia, or Entire Absence of the Spinal Cord, is associated 
with a condition of anencephaly, and the absence of both may be designated 
as amelyencephaly. In some instances there is tolerably normal develop- 
ment of the cord, however, while every trace of the brain may be wanting. This 
defect of the spinal cord is in some instances a simple form of agenesis, in 
other cases a start has been made in the development of the central nervous 
system, but an unusual accumulation of cerebro-spinal fluid at a later period 
of fcetal life has destroyed the neural axis. In forms of amyelia there is at 
times also a defective development of the vertebras. If the defect is due to a 
spinal dropsy we are able to trace, after the birth of the child, the vertebral 
canal and the spinal membranes which are distended by the excessive ac- 
cumulation of fluid. 

Atelomyelia is the term by which the older authors designated a par- 
tial lack of development, such as the entire defect of the pyramidal tracts, or 
a slight defect in the substance of the cord which occurs at the site of the 
spina-bifida. A similar defect is at times observed in the lower portions of 
the spinal cord, which is generally associated with some congenital defect in 
the development of the lower extremities. The only form of atelomyelia 
which is of any practical significance, is the one referring to the defective de- 
velopment of the pyramidal tracts, and that has been considered in the chap- 
ter on hereditary spastic paralysis. 

Asymmetry of the Cord has been described in a few instances. It is 
probably due to defective development of some of the systems of the spinal 
cord, particularly of the pyramidal tracts on one side of the cord, and not on 
both. 

Heterotopia is a condition which has aroused considerable interest of 
late. It denotes malposition of the gray matter of the cord ; parts of which 
are found scattered among the systems of white fibres, either in the lateral 



DEFECTIVE DEVELOPMENT OF THE CORD. 387 

columns or even in the posterior columns. Van Gieson has shown that het- 
erotopia can easily be caused by careless manipulations of the spinal cord 
during post-mortem removal from the body. He throws some doubt upon 
the existence of any such condition as heterotopia during life. While it may 
be conceded that many of the cases that have been reported as heterotopia, 
were, in all probability, artificially produced, we cannot deny the occasional 
existence of such a condition : for it has been found when the spinal cord has 
been most carefully removed, and, furthermore, if it were invariably an arti- 
ficial condition, we would surely find it in many more spinal cords than is 
actually the case. 

- A small cord or microinegaly has often been noted ; in cases of hereditary 
cerebellar ataxy for instance. A normal adult cord has a diameter of 6 to 9 
mm. in the dorsal vertebrae; 8 to 11 mm. in the upper cervical; 15 mm. in 
the cervical, and 12 mm. in the lumbar enlargement. 

Diplomyelia denotes a double cord. This condition is at times found 
in monstrosities of various kinds, but occasionally in children who have ex- 
hibited no spinal symptoms during life. It is due to a defective develop- 
ment of the spinal cord, and is to be attributed chiefly to an abnormal dilata- 
tion of the central canal. In the first months of fcetal life the central 
canal is relatively wide, and if normal development takes place the canal 
becomes smaller and smaller as time goes on. If this retraction does not 
take place, the substance of the cord remains divided and we practically 
have a double structure. This division is, however, never complete, except 
in a few cases 'associated with anencephaly. This diplomyelia need not in- 
volve the entire cord. At times it is restricted to a few segments only. A 
double central canal is not a great rarity. It denotes an arrest in the normal 
development of the cord and as a rule involves only a part of the medulla 
spinalis. The two canals lie side by side. The relation which these defects 
of the central canal bear to syringo-myelia is very evident and need not be 
dilated upon. But it will be remembered that there is some doubt whether 
syringo-myelia represents an arrest in the development of the spinal struct- 
ures, or a destruction of parts that have been normally developed. If the 
ependymal lining of the central canal is entirely normal the syringo-myelia is 
likely to be a congenital condition and not one due to disease. The disten- 
tion of the central cavity has been described as hydrorrachis interna. In 
contradistinction to this we have the condition of 



External Hydrorrachis and Spina Bifida. — This 
is by far the most important of all the congenital anomalies 
of the spinal cord, and is by no means a great rarity, oc- 
curring in about one of every thousand cases. Spina 
bifida denotes a condition in which there is an unusual ac- 
cumulation of serous fluid in the vertebral canal either 
between the pia mater and the arachnoid or between 
the arachnoid and the dura. Associated with this in- 



388 



THE NERVOUS DISEASES OF CHILDREN, 



crease of fluid is a clelt in the vertebrae due to an ab- 
sence of the vertebral arches, and in some instances the 
bodies of the vertebras are wanting. If there is no external 
prominence the condition is called spina bifida occulta. 
Recklinghausen insists that the dura also is cleft in the ma- 
jority of cases. Through this cleft in the vertebrae the 
spinal membranes or the cord may protrude, and accord- 
ing to the parts protruded we can distinguish three kinds of 
spinal hernia : First, meningocele ; second, meningomyelo- 
cele ; third, syringo-myelocele. The first two are much 
more common than the last named. The annexed figures 
will illustrate the condition better than any verbal descrip- 



o b ca 





ftbca 




Fjg. ioi.— Meningocele, Meningomyelocele, and Syringo-Myelocele. a, vertebral 
walls ; b, cord ; c, membranes. (Dana.) 



tion would. In all of the cases the wall of the sac is lined by 
the arachnoid, but not invariably by the dura. The pia is 
not part of the sac-wall unless there is also an internal 
hydrorrhachis or hydromyelus. In some of them the spinal 
cord as well as the nerve-roots protrude into the sac. In 
others again only a few nerves are found in it, the tumor 
contains cerebro-spinal fluid, some connective tissue, and, as 
a rule, considerable fat. 

Etiology. — Spina bifida is caused by defective closure 
of the vertebral arches, formed from the mesoblast. 

This is a primary defect in development, and not caused 
by dropsy of the spinal cord, as was once supposed to be 
the case. 

Normally the vertebral column is closed from above 



DEFECTIVE DEVELOPMENT OF THE CORD. 



389 



downward. It is natural, therefore, that the defect should 
be found most frequently in that part (the lumbar seg- 
ments) which is the last to close. The external tumor is 
generally to be seen in the region of the second to fifth 
lumbar vertebrae. There is distinct fluctuation in the ma- 
jority of cases ; the size may vary from that of an egg to 

the size of a child's head. The 
skin of the tumor may be normal ; 
in some cases it is thinned out, 
particularly if the tumor grows 
rapidly. Ulcers are occasionally 
formed ; the skin may be ruptured, 
but the dura continues to act as 
the wall of the sac. In many cases 
there is a narrow opening between 
the sac and the spinal canal. At 
times there is a complete constric- 
tion at this point, giving rise to 
an ordinary cystic formation which 
may contain some of the spinal 
structures that have been separated 
from the main contents of the spi- 
nal canal. 

Symptoms. — The chief symp- 
toms are those of increased press- 
ure in the spinal and cranial cavi- 
ties, and such as point to a direct 
interference with the functions of 
the spinal cord. Children with 
spina bifida are often poorly devel- 
oped, and mentally deficient. If 
the child lives long enough it pre- 
sents spastic or flaccid paralysis of 
the lower extremities with anaesthesia and with or with- 
out atrophy. Talipes varus is frequent. Remak described 
a form of club-foot associated with spina bifida, differ- 
ing from congenital club-foot. The child is late in learn- 
ing to walk and has little or no control over the 
sphincters of the bladder and rectum. Ectopia of the 
bladder and other viscera, defective development of the 




Fig. 102.— Child of Seven Years, 
with Spina Bifida and Deform- 
ity of the Feet. 



390 THE NERVOUS DISEASES OF CHILDREN. 

lower extremities, are frequent complications of spina 
bifida. 

Diagnosis. — The condition is easily recognized ; it is to 
be differentiated from congenital and other tumors which 
occur often enough in the lumbo-sacral region and may be 
connected either with the substance of the cord or with the 
spinal membranes. 

Fibroma, sarcoma, myxoma, hygroma, and echinococ- 
cus cysts are the forms of tumor which occur common- 
ly in this region. It is of importance to decide whether 
the sac contains nerve structures or fluid only. An explo- 
ratory puncture with a needle (attached to an electric bat- 
tery) will help to clear up the point. 

The course and prognosis of spina bifida will vary much 
according to the extent of the hernia ; in some instances, 
the sac ruptures before or very soon after birth ; these 
cases are invariably fatal. The danger of rupture with 
subsequent purulent meningitis is great in all cases. If the 
opening of the sac is closed, the child may continue to live 
for a long period, but it is after all rare for a person with 
spina bifida to live to middle life. If the sac contains nerve 
structures, the prognosis is less favorable than if it contains 
fluid only. 

Treatment. — Much ingenuity has been exercised by 
surgeons in the attempt to cure these cases. Compres- 
sion and ligature (of the pedunculated tumor) have been 
practised in former years, but the results have been disap- 
pointing. Ahlfeld was probably the first to cure a case of 
this sort by surgical procedure. Of late years surgeons 
have become much bolder. Bayer and Hildebrand, Schede, 
Bergmann, and others have reported a number of successful 
cases. The meningoceles and the meningo-myeloceles are 
considered fit cases for operation if the child's health is 
good, and if there are no serious complications such as 
hydrocephalus, deformities of the extremities, ectopia of 
the bladder and the like. In case the exact contents of the 
sac are in doubt, the X-rays may be used. A free incision 
of tke sac rs always to be preferred to injections into it of 
iodine and similar fluids. 



DISEASES OF THE BRAIN. 
CHAPTER XXIII. 

MENINGITIS AND ENCEPHALITIS. 

Inflammation of the coverings of the brain, particu- 
larly of the pia (lepto-meningitis), is at times secondary 
to disease of the brain proper. More often it represents 
the first and most important factor of a morbid process. 
The anatomical designation, meningitis, may be applied 
to a number of different diseases. The symptoms are very 
much the same in all these forms of disease, and such varia- 
tion as occurs depends in part upon the intensity of the 
process, and in part upon the topographical distribution of 
the disease. It is natural, therefore, to expect that the symp- 
toms of a traumatic or of an idiopathic meningitis, which, as 
a rule, involves the convexity of the brain, will be different 
in certain respects from those of a tuberculous meningitis, 
which involves the base of the brain ; not forgetting that 
the tuberculous process per se is responsible for the severity 
of the symptoms, and the rapid course which that disease 
runs. 

We shall distinguish between — I., Simple acute menin- 
gitis; II., Tuberculous meningitis; III., Epidemic cerebro- 
spinal meningitis, and IV., Meningitis due to various 
causes. 

SIMPLE ACUTE MENINGITIS. 

This form of disease occurs after many infectious dis- 
eases (pneumonia, ulcerative endocarditis, erysipelas, etc.); 
after slight or severe injuries, after insolation, and in con- 
nection with acute nephritis. The symptoms of acute men- 
ingitis are very similar to those observed in all the forms, 
except that they point to an involvement of the convexity 



392 THE NERVOUS DISEASES OF CHILDREN. 

much more frequently than to an involvement of the base. 
The disease begins, as a rule, with a feeling of malaise, with 
vertigo, with nausea and vomiting. These symptoms may 
remit for a few days or even a week, or they may progress 
continuously until the headaches become distressing and 
the vertigo so intense that the child cannot stand, while the 
nausea and vomiting are repeated at frequent intervals. 
The vomiting has in most instances the true cerebral char- 
acter (projectile vomiting). It occurs both after taking 
food and independently of this. If the vomiting under 
these circumstances is associated with a clean tongue, its 
cerebral origin becomes all the more probable. But too 
much stress should not be laid upon this one point, for gas- 
tric disturbance is so common in children, and so often pre- 
cedes the onset of severe cerebral disease that the presence 
of a coated tongue need not suggest the improbability of 
cerebral disease. By degrees the child becomes listless and 
apathetic, it begins to be drowsy, and sleeps a great deal, 
and its existence is taken up with sleeping and crying. A 
convulsive seizure often occurs during this stage. The child 
becomes irritable, restless, shuns the light (photophobia). 
The temperature varies between ioi° and 104 F.; the pulse 
is rapid at first, then becomes irregular and slow ; the 
pupils are contracted for a time, later on dilated. The 
child grows more and more unmanageable until the apathy 
deepens, and it finally passes into a comatose condition. 
Before the condition of coma is reached in the vast major- 
ity of the cases slight rigidity of the neck sets in, and the 
upper as well as the lower extremities often exhibit marked 
spasticity. The deep reflexes are, as a rule, increased. The 
abdomen is retracted ; the bowels are constipated, and if 
the child is in a deeply comatose condition all efforts at 
feeding it, or at making it take the breast, are entirely un- 
availing. 

According to the severity of the disease these symptoms 
will be developed in a shorter or longer period of time, but 
as a rule a week from the onset is quite sufficient for a full 
display of the symptoms. To the symptoms noted above 
we may add the loss of vesical and rectal control, retention 
of urine, or involuntary micturition and defecation. Vaso- 



MENINGITIS. 393 

motor disturbances are marked ; taches cerebrales occur in 
the disease, but have no diagnostic significance. In some 
cases ocular palsies (strabismus, ptosis) may be present, and 
the optic neuritis may give rise to temporary or permanent 
blindness. A monoplegic or hemiplegic paralysis having all 
the symptoms of a cerebral palsy may be developed. If the 
disease takes an unfavorable turn all the symptoms increase 
in severity, the rigidity of the neck becomes extreme ; opis- 
thotonos is developed ; the blindness continues ; the coma 
is deepened ; respiration becomes irregular, the Cheyne- 
Stokes type becoming more marked until respiration ceases 
altogether. If the patient is to pass on to recovery, the 
symptoms become stationary for a time after the comatose 
condition has been reached ; then the rigidities are lessened, 
the optic neuritis may recede, and from day to day there are 
evidences of returning consciousness. 

Etiology. — The occurrence of acute meningitis in as- 
sociation with acute infectious diseases will be referred to 
again. " Idiopathic meningitis " has been held by many to 
be a cover for our ignorance. There is no doubt, however, 
that meningitis may be developed without assignable cause 
in a child that comes of healthy parents, and that has itself 
enjoyed perfect health up to the time of the onset of the 
disease. One variety may be attributed to the effect of in- 
tense heat (sunstroke). It is quite likely that a considerable 
number of the so-called idiopathic forms are due to injuries, 
the traumatic factor bein^ so slight at times that it does not 
receive the attention which it merits. 

About ten years ago a child was seen in my clinic that had fallen from its 
crib, a fact which the mother mentioned quite casually. When the child was 
first examined it presented the typical symptoms of the first stage of a gen- 
eral convexity meningitis. It was slightly stuporous, unable to stand or sit 
alone, the head was stiff, and it had had occasional spells of vomiting. This 
stuporous condition soon passed into one of deep coma, the child lost ground 
rapidly, never exhibiting, however, any cranial-nerve symptoms. After a 
period of at least six weeks, during which time the child was more or less som- 
nolent, a favorable change set in. consciousness began to return, and the child 
recovered fully from what was evidently a simple traumatic meningitis. 

Morbid Anatomy and Pathology. — Simple acute 
meningitis is characterized by an inflammatory condition 



394 THE NERVOUS DISEASES OF CHILDREN. 

of the pia mater, which is, as a rule, attended by slight in- 
flammation in the dura and in the gray matter of the brain. 
In contradistinction to other forms this inflammation is of 
a serous, or at least of a non-purulent character. There is 
an increase of cerebro-spinal fluid, the arachnoid may ap- 
pear a trifle opaque, while the substance of the brain is dis- 
tinctly cedematous, and even watery. The ventricles are 
distended, and there may be a condition corresponding to 
an acute hydrocephalus. The pia of the convexity of both 
hemispheres is the part most extensively diseased, the pia 
of the base often being entirely free from all disease, though 
a slightly increased exudation of lymph may be apparent 
in the interpeduncular space. 

On microscopical examination the blood-vessels of the 
pia are generally found to be slightly engorged, and an ex- 
travasation of white blood-corpuscles is found in the vicin- 
ity of the blood-vessels. If the disease has lasted for any 
considerable length of time, the pia and the cortical sub- 
stances have become agglutinated so that the pia cannot 
be removed without tearing the outer layer of gray matter. 

Diagnosis. — The diagnosis of simple acute meningitis 
rests upon the recognition of the symptoms common to all 
forms of meningitis. These are headache, vomiting, coma, 
and convulsions ; irregularity of the pulse and unequal pupils. 
It is well to note also the absence of those factors which 
accompany the graver forms of meningitis (high fever, ba- 
silar symptoms, rapid emaciation, and a rapid increase in 
all the symptoms). The differential diagnosis will not be 
an easy one, and sometimes a positive diagnosis cannot be 
reached until the disease takes an unexpectedly favorable 
turn, or until the patient is seen upon the post-mortem 
table. But the mistake that is most frequently made is that 
the meningeal symptoms accompanying many acute infec- 
tious diseases are at once pronounced to be the symptoms 
of true meningitis. This diagnosis has, to the author's 
knowledge, been made in the first stages of typhoid fever, 
and in measles and scarlet fever beginning with convulsions ; 
the error is common in otitis and in infantile spinal paraly- 
sis that is attended by convulsive seizures and high fever. 
It is well to remember that though these symptoms resem« 



MENINGITIS. 395 

ble those due to meningitis, they are frequently associated 
with other diseases, and that no physician should be in a 
hurry to diagnosticate meningitis, pure and simple, unless 
other diseases can be safely excluded. 

Course and Prognosis. — The course of a simple menin- 
gitis will almost invariably cover a period varying from four 
to twelve weeks, or even longer. In those cases that do not 
tend to recovery the symptoms gradually become intensi- 
fied, respiration becomes more and more difficult, and after 
dragging along for some weeks, now and again yielding a 
hope of recovery, the child finally succumbs from mere ex- 
haustion, from some intercurrent disease, such as bronchitis 
or pneumonia, or from the effects of cystitis, bedsores, and 
the like. 

In those cases that recover after the coma has lasted for 
a period varying from one to three or four weeks, signs of 
returning consciousness are noticed, the child opening its 
eyes, voluntarily looking around, again taking hold of the 
bedclothes, of the hand of the nurse or mother, and so on. 
By degrees the sight improves, hearing, if diminished or in- 
creased, becomes normal, the child begins to take its food 
properly, and from week to week a distinct improvement is 
noticeable, until all the symptoms have disappeared. But 
not all of the cases that recover end thus fortunately. The 
disease does not necessarily kill, but it often leaves distinct 
traces behind. Not a few of those who are permanently 
blind owe their misfortune to a meningitis in early years. 
Permanent paralysis and contractures of one or more ex- 
tremities are attributable to the same cause, and defective 
speech, and, above all, a defective intellect are very often the 
unfortunate outcome of meningitis in early life. Such de- 
formities and defects do not, however, result as frequently 
from simple meningitis as from the severer types, from 
which recovery is, on the whole, much rarer. 

Treatment. — In every form of acute meningitis, what- 
ever its origin may be, I am in favor of adopting the fol- 
lowing procedure : 

First, keep the patient absolutely quiet, and in a semi- 
darkened room, and secure the services of a careful and in- 
telligent nurse. Give the child an efficient purgative ; 



396 THE NERVOUS DISEASES OF CHILDREN. 

none will do better than calomel, which has attained a cer- 
tain dignity in all diseases of the central nervous system. 
Place an ice-bag over the convexity of the skull, or on the 
nape of the neck if the symptoms point to the involvement 
of the basilar portion. Though we cannot claim any direct 
therapeutic effect it will do no harm, for it will at least 
help a little to reduce the general rise of temperature, and 
is useful as the first point of attack in treatment. Place the 
child at once, according to its age, upon moderate doses of 
the bromide and iodide of sodium (three to five grains of 
each every four hours). Give these in simple water, or 
in milk. This treatment may well be persisted in for a few 
days. If no effect is observed, while it is well to continue 
the salts of sodium, other more energetic measures may be 
employed in addition. Among these I would place, first, al- 
ternate lukewarm and cold douching of the nape of the neck 
and the upper portion of the spine, and inunctions of some 
form of mercury, either of the ten per cent, oleate of mer- 
cury or of the unguentum hydrargyri. Inunctions should 
be performed by the nurse or the mother, and done so 
thoroughly that the mercury disappears into the skin of 
the patient. 

For purposes of diagnosis, as well as for therapeutic 
reasons, lumbar puncture may be attempted ; in those cases 
in which the symptoms point to greatly increased intra- 
cranial pressure, it will be best to tap the spinal canal on 
successive or on alternate days, withdrawing only about 
10 c.c. at any one time. 

After the patient has made a fair recovery, the general 
condition of the system will need looking after, and a thor- 
ough course of cod-liver oil, of malt, or of iron, will be quite 
in order, and for some months after recovery from this 
severe disease, the child should be kept free from all ex- 
citement and from all mental and physical fatigue. A 
restful out-of-door life is the very best conclusion to this 
method of treatment. To patients who exhibit the least 
tendency to paralysis or contracture, massage should be 
given as soon as the acute symptoms have disappeared, and 
electric treatment should be applied to the parts that are 
distinctly paralyzed. 



MENINGITIS, 397 



TUBERCULOUS MENINGITIS 

Tuberculous meningitis is by far the most frequent form 
of meningeal disease in children, if we except the ravages 
in recent years, and in this community, of the epidemic 
form of cerebro-spinal meningitis. 

The disease itself was first recognized by Robert Whytt, in 1768, and 
since that time has been the subject of innumerable articles ; but every point 
regarding the disease is now so thoroughly known that we can sum up its 
chief characteristics without referring to individual authors. Since Koch's 
discovery of the bacillus of tuberculosis there has been no doubt of the 
microbic origin of tuberculous meningitis. According to Holt's statistics, 
twenty-five per cent of all deaths from tuberculosis in children were due to 
meningitis. This form of meningitis is always secondary. 

The disease occurs in children much more frequently 
than in adults; while it is rare in young infants, it is more 
frequent in children between the ages of six months and 
four years. In families in which there is a distinct heredi- 
tary taint of tuberculosis or scrofula, children that have 
been apparently well are suddenly attacked by this malig- 
nant disease, and healthy children in families in which every 
hereditary predisposition is wanting, or, at least, denied, 
are also affected. But in the majority of instances the chil- 
dren attacked have been weak and feeble ; many of them 
have suffered from chronic intestinal troubles, from swell- 
ings of the glands, from nasal and aural catarrhs, in short, 
from those conditions from which we may safely argue a 
tubercular infection. A chronic laryngitis or bronchitis, or 
a swelling of the bronchial glands, which may not have 
given rise to any special symptoms, is often the precursor 
of tuberculous meningitis. 

Onset. — In contradistinction to other forms of meninsfi- 
tis, that dependent upon tubercular infection comes on in a 
very insidious fashion. The child first loses its brightness 
and cheerfulness, and complains of an occasional headache, 
and slight nausea; a vomiting spell may occur. The pulse 
is generally rapid. These complaints almost invariably lead 
to the suspicion of slight gastric disturbance, which, fortu- 
nately, is quite often the case ; but every physician will 



398 THE NERVOUS DISEASES OF CHILDREN. 

do well, whenever this series of phenomena occurs, to be 
watchful, and to give notice that he should be informed if 
the symptoms do not promptly disappear. There may be 
a marked remission or great improvement for a few days ; 
then a change takes place, the nausea becomes more frequent, 
the headache more intense, and the vomiting spells are a 
daily occurrence. In this way a week or more may pass, the 
physician and parents hoping that all the symptoms may 
subside. At the end of about a week little doubt remains of 
the significance of the condition, for if a child is afflicted 
with a tuberculous meningitis the headaches become intense 
and persistent, the child gives the short hydrocephalic cry, 
and by degrees becomes somnolent ; slight rigidity of the 
neck is observed, the pulse has become slower, and the child 
shows every sign of serious illness. Aphasia may be an early 
symptom ; and all signs point to an intense general brain dis- 
turbance, and to the localization of the disease chiefly at the 
base of the brain, from which the various cranial nerves issue. 
The temperature is subject to great variations, and during 
the first few weeks it does not, as a rule, rise above 103 F., 
but during the last week it may reach 105° F., and during 
the terminal stages may reach 106 F., or even higher. In 
one case, an hour before death, I recorded a temperature of 
107 F. This is supposed to be due to a paralysis of the 
heat-regulating centres. Respiration is not seriously inter- 
fered with, as a rule, until the child enters upon the ter- 
minal period, during which stage the breathing becomes 
irregular, often of the typical Cheynes - Stokes type, and 
cyanosis is added to the host of other symptoms. 

As we examine the child from head to foot, we are 
apt to find a variety of symptoms. Rigidity of the neck, 
with or without opisthotonos, and excessive painfulness on 
every passive movement of the head or trunk. The major- 
ity of the patients present distinct convergent strabismus, 
due, as a rule, to a paralysis of one or more of the ocular 
muscles. The pupils are unequal and dilated, contracting 
very sluggishly to light, and their reaction during accommo- 
dation can, of course, not be tested in consequence of the 
comatose condition. The conjunctival reflex is lost at an 
early period, and in consequence of diminished movement 



MENINGITIS. 399 

of the eyelids the cornea becomes cloudy easily. Degluti- 
tion can be carried on only imperfectly, and during an ef- 
fort to open the mouth or to perform chewing motions, a 
trismus is very apt to set in. A further examination of the 
head may reveal a paralysis of some of the branches of the 
facial, the paresis of these muscles being, at times, unilat- 
eral and at other times bilateral ; but the paresis is evi- 
dently due to involvement of the nerves at the base, and if 
an examination is made to bring out this special point, the 
electrical reactions may be found altered in keeping with 
this special localization of the lesion. 

An examination of the eyes will disclose a hyperasmic 
and swollen condition of the papillas. In some instances 
there may be a typical optic neuritis, single or double.* 

The abdomen is retracted, taches cerebrales are easily 
produced (not pathognomonic). 

The upper and lower extremities may be paralyzed to 
a greater or lesser degree ; the exact amount of paralysis 
cannot always be easily determined on account of the en- 
tire absence of voluntary action, and because the physician 
is unable to make satisfactory tests for this special point. 
While the paralysis may be of sufficient interest as illus- 
trating the exact distribution of the meningeal process it is 
of very little practical importance, and loses in value as 
compared with the other and more serious symptoms. The 
paralysis may be hemiplegic; in some forms it is bilateral 
or irregular. The cutaneous reflexes are diminished, often 



*Much has been made of the presence of tubercles in the choroid, and it has been 
claimed by many that the existence of these tubercles is one of the most important 
symptoms of the earlier stages of the disease. The truth of this cannot be gainsaid, 
but as a matter of practical experience it must be admitted that in many cases of un- 
doubted tubercular meningitis the expert oculist does not find tubercles, and that in 
those very cases in which the discovery of such a tubercle would have helped to de- 
termine the exact character of a meningitis, the tubercles cannot be seen, although 
later post-mortem examination leaves no doubt of the tubercular character of the 
process. While we may regard the presence of tubercles, therefore, as a valuable 
corroborative symptom, a failure to detect them should not be allowed to disprove the 
diagnosis if other symptoms would seem to point to the tubercular nature of the 
trouble. Dr. R. L. Randolph has examined thirty-five cases of meningitis of all kinds 
with the ophthalmoscope, and found the fundus normal in seven patients. The optic 
disks were usually congested, with the retinal vessels distended and remarkably tortu- 
ous. In three cases the entire eye was normal, and these three patients recovered. In 
the four fatal cases with normal fundus some other ocular symptoms were present. 



400 THE NERVOUS DISEASES OF CHILDREN. 

lost. The deep reflexes in the upper and lower extremities are, 
as a rule, increased. Kernig's symptom is generally present. 

Epileptiform convulsions are not rare, both at the onset 
of the disease and during later stages. These convulsions 
are, as a rule, general in distribution, and not of the Jack- 
sonian type. If the latter form should prevail, there would 
be good reason to infer that the cortex is diseased as well 
as the base of the brain. Considering the fact of the ex- 
istence of so-called epileptiform centres in the pons and 
medulla, the wonder is not that such convulsions occur, but 
that they do not occur much more frequently. In the 
terminal stages of the disease convulsions become more 
frequent, as they do in many other brain diseases; and in 
tubercular meningitis the frequent occurrence of convul- 
sions, with a rise in temperature, may be taken to be the sign 
of the approaching end. In the terminal period, too, the paral- 
ysis becomes complete, the pupils are dilated, the tongue dry 
and furred and the temperature may fall to 93 or 94 F., 
until an ante-mortem elevation of temperature begins. 

All' the phenomena are remarkably persistent during the 
course of this disease. There are at times slight remissions 
in the ocular palsies, but after the symptoms have once 
been fully developed, they remain very much the same to 
the end. The changes which take place are these : Respira- 
tion becomes irregular, at times intermittent, the pulse 
grows feebler and slower, deglutition more and more diffi- 
cult, and the child dies from paralysis of the cardiac and 
respiratory centres. 

Morbid Anatomy and Pathology.— Every one who 
has had an opportunity to remove the brain from cases of 
tuberculous meningitis is surprised by the few changes 
found in this organ. In the great majority of autopsies, on 
the removal of the calvarium there is very little evidence 
of any active process on the convexity. In some brains the 
pial vessels are much congested, the sinuses are filled with 
clots that have evidently been formed only a few days be- 
fore death, and the hemispheres in general present a more 
or less cedematous appearance. 

Minute tubercles are noticed along the distribution of 
some of the larger pial veins on the convexity. The chief 



MENINGITIS. 40I 

changes cannot be noted until the brain has been removed 
from the skull. At the base of the brain the character of 
the disease is easily recognized, the pia is cloudy, and in 
some places bulging because of the accumulation of fluid 
underneath. This is particularly noticeable in the interpe- 
duncular spaces, in which the tubercles are, as a rule, most 
freely developed. These tubercles, often no larger than a 
pin's head, may be scattered throughout the entire pia from 
the optic chiasm to the pons, medulla, and spinal cord. 
The presence of tubercles is not always established at the 
time of the autopsy, but this need not militate against the 
proper diagnosis, for it is a fact, well proven, that in un- 
doubted tubercular diseases we may have an involvement 
of the pia without tubercles, and occasionally the presence 
of tubercles with but few signs of an inflammatory process. 

Pathology. — Of the pathology of tubercular meningitis 
little need be said, as it belongs to the order of infectious 
disorders, and the disease is, in almost every instance, a part 
of a general tubercular infection. The tubercular form is, 
moreover, rarely a primary affection, although it sometimes 
occurs in children who have been apparently healthy. A 
thorough post-mortem examination reveals quite regularly 
distinct foci of disease in the lungs, in the mesenteric glands 
from which the infection in all probability took its start. The 
invariably fatal issue must be attributed in part to the gen- 
eral effect of the tubercular poison and its ravages in other 
organs ; for the remarkably slight changes in some of the 
cases that have taken a rapid course are the surprise of 
every pathologist. A few small tubercles, without much 
exudation, even though they be in the vicinity of the pons 
and medulla, can hardly be considered a sufficient cause of 
death, whereas the toxines circulating in the blood may have 
been the initial cause of the paralysis of the vital centres. 

Diagnosis. — The difficulties of a differential diagnosis* 
are not limited merely to the period of onset, during which 

* The general practitioner is apt to attach too much importance to the general 
symptoms, and too little to the local (basilar) symptoms. The pulse, the tempera- 
ture, the condition of the abdomen, may leave the diagnosis in doubt, but an acute 
ocular palsy, however slight, or an incipient optic neuritis, will indicate the true nature 
of the trouble. 



402 THE NERVOUS DISEASES OF CHILDREN. 

time the most experienced physician may well be in doubt 
as to the true character of the disease. But the more diffi- 
cult question arises later on, whether the disease be a simple 
meningitis, whether it be of tubercular origin, or whether 
it represents an epidemic form. In order to determine this, 
it is best to keep in mind the antecedent history of the 
child ; a history of tubercular trouble in the family, or of 
an early scrofulous or chronic catarrhal condition would 
naturally prejudice the physician in favor of a diagnosis of 
tubercular trouble, but everyone has experienced curious 
surprises in this respect, for children of tubercular ances- 
try may have a simple meningitis which is recoverable, or 
they may be afflicted with the epidemic form. The diag- 
nosis will be confirmed by the presence of tubercle bacilli 
in the spinal fluid, or according to French authors, by the 
presence of mononuclear lymphoid cells. The absence of 
bacilli need not militate against the diagnosis. In addition, 
it is fairly safe to infer that cases of tubercular meningitis 
invariably present basilar symptoms, whereas the symptoms 
pointing to involvement of the convexity only, occur more 
frequently in the other forms. 

Course and Prognosis. — Tuberculois meningitis runs, 
as a rule, a course varying between three and six weeks, 
though the time may be extended a little if the premonitory 
period is taken into account. In the more virulent forms 
death may occur at the end of the first week, or in the 
course of ten or twelve days ; and in these forms I have 
often found only slight post-mortem changes, from which 
we may infer that the general toxine poisoning has been of 
much more consequence than the local deposit at the base 
of the brain. As for the prognosis, no one need hesitate to 
say that it is absolutely bad; but in making such a prognosis 
the most experienced physician will do well to remember 
that this diagnosis is never quite as certain as is the prog- 
nosis based upon it. There is grave doubt whether cases 
of tuberculous meningitis ever recover. 

Henoch, and Rilliet and Barthez, record two cases in which death ensued 
from a second attack, occurring some years after the first ; but even here there 
is room for doubt as to whether the first attack of meningitis was truly tuber- 



MENINGITIS. 403 

cular in character. Pollitzer reports a case of a child which survived three 
years after an attack of basilar meningitis ; at the autopsy he found, at the 
base, the distinct evidences of an old exudation over the pons, which was in 
all probability of a tuberculous nature. Freyhan found the tubercle bacilli in 
the cerebro-spinal fluid of a patient who recovered. 

Oppenheim has reported six cases of a localized tuberculous meningo-en- 
cephalitis of the Rolandic areas in children, simulating brain tumor, ending in 
recovery. The anatomical proof is wanting, but the author's hypothesis is 
worth considering. 

Treatment. — The patient should be put as quickly as 
possible in a quiet, darkened room. Administer calomel at 
once, in a sufficient dose to effect a very copious discharge 
from the bowels. If the child will tolerate it, put an ice-bag" 
over the nape of the neck ; or, if the ice-bag is unpleasant, 
ordinary cold applications can be tried. As soon as the 
bowels have been moved give the iodides, the bromides, 
or mercury ; employ those therapeutic measures which we 
have discussed in connection with other forms of menin- 
gitis. 

From the very beginning, too, observe the cardiac and 
respiratory functions, and give mild cardiac stimulants. 
The best are small doses of digitalis and caffeine. 

Special attention should be paid to the feeding of the 
child. Feeding by the spoon and giving the food in very 
small quantities at a time is the only proper method. If 
great care is not exercised the liquids may flow into the 
trachea and produce very uncomfortable symptoms, with 
the possible result of complicating pneumonia. According 
to the condition of the child the physician should exercise 
his judgment, and remember that loss of sleep and lack of 
quiet are often much more harmful than lack of food, and 
every child suffering from any form of meningitis should 
be given ample time for quiet sleep. The old habit of in- 
sisting on half-hourly, or even hourly, feeding is not to be 
commended in these cases. 

Unfortunately there is little reason with the majority of 
patients to change this method of treatment, for whatever 
method be employed the results are equally disastrous; but 
a persistent effort should be made, and the attempt to con- 
quer the disease should not be given up until the child be- 
gins to decline rapidly, until it fails to swallow food, for 



404 THE NERVOUS DISEASES OF CHILDREN. 

from that time on medication will do very little good. Per- 
sistent administration of medicines, of nutrient enemata, 
and the like, beneficial as they may be in other diseases, in 
these simply tend to prolong the agony of the child, and of 
the careworn parents or relatives. Lumbar puncture is of 
no avail in this form of meningitis, and, after the diagnosis 
has been established, should not be repeated. 

Surgical interference has been attempted in tubercular meningitis. Ord 
and Waterhouse have trephined a case diagnosticated as tubercular menin- 
gitis, and have drained the subarachnoid space; * the child recovered. There 
is reason to doubt the tubercular character of the disease in this case, but the 
relief after the operation was so marked that the propriety of an operation 
can be entertained before the child is exhausted, if pressure symptoms are 
extreme and the character of the meningitis is doubtful. A recent author 
(Hirschberg) believes that death is due, not to the tubercles, but to intracra- 
nial compression. We have stated on a preceding page that the increase in 
intracranial fluid is often very slight indeed. The trephine in Ord's patient 
was applied midway between the external occipital crest and mastoid process. 
The operation has been attempted in this city on an undoubted case of tuber- 
cular meningitis and the child died very shortly thereafter. 



EPIDEMIC CEREBROSPINAL MENINGITIS.f 

This special form of meningitis has attained a sad dis- 
tinction in many countries. In America its ravages have 
been very much greater than in Europe.^ In New York 
City serious epidemics occurred in the winter and spring 
of 1904 and 1905. It belongs to the category of infectious 
fevers. The microbic origin of the disease has recently 
been established beyond peradventure, though it seems to 
be still a matter of doubt as to whether one or more micro- 
organisms bear an etiological relation to it. 

* Such drainage could be effected quite as readily by a lumbar puncture according 
to Quincke's method. 

tSynonyms: " Spotted fever," " cerebro-spinal fever," " typhus petechialis," " febris 
nigra," and "malignant meningitis," are the common synonyms, all of them indicating 
the infectious and grave character of the disease 

% At this present time (1905) serious epidemics of " spotted fever " have been reported 
from various parts of Continental Europe. In eastern Prussia the disease appeared in 
its most virulent form ; and if the earliest statistics are reliable, the mortality rate is 
about the same as in American cities. In spite of " commissions " that have been ap- 
pointed, no sovereign remedy has been discovered. 



MENINGITIS. 405 

It is the accepted opinion of the day that this grave disease is due to 
infection by the diplococcus intracellularis (Weichselbaum, Jaeger, Heubner, 
Osier, Koplik, Holt, and others). Some sporadic cases have been shown to 
be due to the micrococcus pneumoniae of Fraenkel, and in some cases, 
resembling the epidemic form clinically, streptococci and other micro- 
organisms have been found. The only just inference is, that various or- 
ganisms may produce cerebro-spinal meningitis, but that the epidemic form, 
and no doubt many of the sporadic cases, are due to the diplococcus intra- 
cellularis. 

The epidemics have varied much in severity, and in each epidemic there 
have been some mild, and an alarming number of most virulent cases. The 
micro-organisms have been found in the nasal secretions, thus suggesting a 
possible point of entrance into the cranial cavity. Among the New York 
physicians reporting upon the latest epidemics are Chapin, Nammack, Manges, 
Koplik, Lambert, Berg, and others. 

The first epidemic of cerebro-spinal meningitis was distinctly recognized 
as such, and well described under the heading of " a malignant non-conta- 
gious fever " by Vieussens in 1805, who described the disease as it appeared 
in Geneva. Thirty-three persons lost their lives during this epidemic ; the 
average duration of the disease was from one-half to five days. A few 
years later epidemics occurred in various parts of Germany, in Holland, and 
in England. Dr. J. Lewis Smith, to whom we are indebted for one of the 
best contributions to our knowledge of this disease, states that the first Amer- 
ican case occurred at Medfield, Mass., in 1806. From 1806 to 1816 it ap- 
peared in various localities both in Canada and in the United States. Be- 
tween 1 816 and 1828 one epidemic occurred at Middletown, Conn., and 
another at Vesoul, in France. In 1833 Naples was visited by this epidemic, 
and the disease did not appear again until 1837, and then various localities in 
France were stricken. The military were chiefly affected by the disease, and 
a very large proportion of those affected died from it. Between 1837 and 
1849 France was the chief seat of the epidemic. In the next ten years al- 
most every part of Europe was visited by the disease. In 1842 another epi- 
demic broke out in the United States, at a distance from the sea-coast, and as 
Dr. Smith says, apparently not by communication from Europe ; but this 
could hardly be maintained with our present views regarding the transmis- 
sion of micro-organisms from one country to another. Epidemics occurred 
in States as widely apart as Alabama and Mississippi, New York and Louis- 
iana. Norway and Sweden were the chief seats of the disease between 1854 
and i860, and since that time scarcely a single city or district has been 
entirely free from the disease. Inasmuch as isolated districts have been af- 
fected, the disease was not supposed to spread in the manner of ordinary 
contagious diseases, but to have been engendered by local conditions, among 
which the massing together of large classes of population in poorly ventilated 
and filthy quarters, as in military barracks for instance, was considered to 
be the most favorable predisposing cause. The disease is now perma- 
nently established in almost every large American city, though it has rarely 
assumed a severe epidemic form. In New York City, from 1866 to 1872, the 



406 



THE NERVOUS DISEASES OF CHILDREN. 



annual deaths from this disease, according to Dr. Smith's statistics, varied 
from eighteen to forty-eight. A very severe epidemic occurred in December, 
1 87 1, and lasted well into the summer of 1872, so that 782 deaths, chiefly in 
children, resulted from cerebro-spinal fever in this city alone. Since that 
time the annual deaths have varied between 97, in 1878, and 461, in 1881. 
An important epidemic, though a small one, was the epidemic of Lonaconing, 
which was most carefully observed by two competent physicians, of which 
an account is given in the article by Flexner and Barker in the year 1894. 
This town is situated in the Alleghany Mountains and contains about five 
thousand inhabitants. A muddy stream which passes through the town, 
receiving most of the sewage, appears to have been responsible for the spread- 
ing of the epidemic. All the conditions, including the overcrowding of 
miners in filthy houses, were favorable to the spread of the disease. 

The disease is common both to man and beast. A serious epidemic 
occurred in New York City in 1871, and was at once recognized as a filth 
disease, for it first affected the horses in the large and overcrowded stables 
of the car and stage lines. A few individuals were soon similarly afflicted, 
but it is doubtful whether the disease was transmitted from the animals to 
the men who were in charge of them. The epidemic which occurred in 1872 
was evidently related to this same outbreak in animals in 1871. Though 
the disease is bred by filth, it may unquestionably also be carried, or at least 
transmitted, to persons, and particularly to children living in excellent hy- 
gienic surroundings, and many of us have seen such cases in the habitations 
of the richest as well as in the families of the working-classes. The disease 
may attack those in good health, but is even more apt to strike those whose 
health has been injured by previous disease or by fatigue. Dr. Smith quotes 
Frothingham as an authority for the statement that in a brigade of the Army 
of the Potomac which was attacked by this epidemic, the men were almost 
exhausted from excessive drilling. 

The disease shows no distinction between the sexes, at least in children. 
Dr. Smith reports 105 cases occurring in his practice, of which 59 were in males 
and 46 in females ; 91 of these cases being in children. While persons of 
every age may be attacked by the disease, it is unquestionably more liable to 
attack children in the earlier years of life than at any other period. It is in- 
teresting to note the statistics for a single year, 1883, as given by Smith for 
New York City': 



Under 


1 vear 


From 


1 to 2 years 


From 


2 to 3 " 


From 


3 to 4 " 


From 


4 to 5 " 


From 


5 to 10 " 


From 


10 to 15 " 



57 
3i 
22 
12 

9 

37 
18 



From 1 5 to 20 years 15 

From 20 to 25 " 

From 25 to 30 " 

From 30 to 35 " 

From 35 to 40 " 

After this scattering cases. 



The New York Health Department records 498 deaths from cerebro-spinal 
meningitis from March 12th to May 21st, 1904. 



MENINGITIS. 407 

Symptoms. — The majority of the symptoms will natu- 
rally resemble those occurring in other forms of meningitis. 
The onset of the disease is characteristically sudden. A 
child in perfect health is suddenly seized with headache, 
vomiting, and either slight rigor or convulsions. In the 
milder cases a few days of malaise and of slight nausea may 
precede the onset of the other symptoms. The first severe 
symptoms are promptly followed by a stupor, which is apt 
soon to deepen into profound coma. I remember the case 
of a child, one year of age, in which a slight headache, 
vomiting, convulsions, rigidity of the neck, deep coma, and 
strabismus all developed within twenty-four hours, and 
death ensued within forty-eight. Severe neuralgic pains 
are frequent in the earlier stages of the disease. The pupils 
are unequal and dilated. The vomiting is of the cerebral 
order and occurs on the first or the second day in the 
vast majority of the cases. The fever is, as a rule, high, 
varying between 103 and 105 F. ; in one rapidly fatal case 
it reached 107 F. a few hours before death on the second 
day. 

The meningeal symptoms are developed in a very much 
shorter period of time than in other forms of meningitis. We 
do not have the slow progress from stupor to coma which 
we find in the tubercular type ; often the coma is deep 
from the very beginning. Delirium may alternate with in- 
tense coma. In some of the cases in which the coma is not 
profound, great restlessness takes the place of the stuporous 
condition. The child is, as a rule, extremely sensitive, even 
hyperassthetic. The slightest touch of any part of the body, 
the mere weight of the bedclothes, is often sufficient to 
elicit shrill cries. This hyperesthesia is explained quite 
readily by the irritation of all the posterior root-fibres by 
the meningeal exudate. Contractions of the various mus- 
cles occur at an early stage of the disease. The head is 
firmly retracted, opisthotonos is distinctly developed, the 
thighs and legs are in a flexed position, and the arms and 
hands may be distinctly contractured. A hemiplegia or an 
alternate form of paralysis, or a monoplegia, together with 
cranial nerve palsies, may be made out in some patients. 
The deep reflexes are exaggerated, and the superficial 



408 THE NERVOUS DISEASES OF CHILDREN. 

reflexes are diminished. Kernig's sign is present. Convul- 
sions are very common. 

The author saw during the spring months of 1904 a number of fulminant 
cases in which the disease ran its entire course within forty-eight hours. In 
such cases the chief symptoms are : Headaches, high fever, rapidly developing 
delirium, rigidity of the neck ; in short, general, not localizing, symptoms. 

The "tache cerebrale " was formerly considered to be a 
rather important symptom of all forms of meningitis ; but 
it has lost its pathognomonic value, as it occurs in many 
other diseases. The skin presents a peculiar mottling in 
the first or second week of the disease, and particularly 
when the temperature is low. Small red points and large 
bluish spots, due to exudation of blood under the surface, 
also appear, and were seen frequently enough to justify the 
term " spotted fever." In European epidemics these peculiar 
extravasations have not been regular^ observed. Herpes 
occurs, and Smith refers to the occurrence of erysipelas ; 
but the latter is evidently entirely independent of the men- 
ingitis; it is a complicating condition, and not in any sense 
a symptom of the epidemic form. 

The organs of special senses are frequently affected in epidemic menin- 
gitis, and possibly more frequently than in other forms. A hyperasmic and 
inflammatory condition of the entire eyeball is a common occurrence. The 
media may become cloudy and the various structures may become adherent 
to one another ; occasionally ulcerations of the cornea and perforation of the 
eye with total loss of vision may occur. According to Knapp, as quoted by 
Smith, the nature of the eye affection is a purulent choroiditis, probably met- 
astatic. In some cases a double optic neuritis occurs, and from this, as well 
as from the inflammatory conditions of the eyeball, total blindness may re- 
sult. Not a few of those who recover from cerebro-spinal meningitis are 
afflicted with permanent blindness as the result of this dreadful scourge. 

The hearing is often as seriously impaired as vision, and severe otitis 
media, ending in suppuration, with perforation of the drum and all its se- 
quences, is a common occurrence. In other instances loss of hearing is evi- 
dently due to more central causes, and is developed only after recovery from 
the main disease. It is unfortunate that the loss of hearing is apt to be bi- 
lateral and complete. According to Smith's statistics from the epidemic in 
1872, about one in every ten patients became deaf. But he states that in the 
milder form of cerebro-spinal meningitis which has prevailed since 1872 the 
proportionate number that has been thus affected has been less, and the 
same may be said with reference to loss of sight. Knapp reports that 



MENINGITIS. 409 

among twenty-nine cases of total deafness occurring after cerebro-spinal 
meningitis only one seemed to give evidence of hearing afterward. 

Morbid Anatomy and Pathology. — We need not 
again insist upon the microbic origin of the disease. If the 
exudate is examined the diplococcus intracellularis or the 
pneumococcus may be found. The chief anatomical charac- 
teristics of the disease are an intense hyperaemic condition of 
the meninges and of the brain, and this may be the sole 
morbid condition if the patient has died in the very early 
stage of the disease. If it has lasted more than a few 
days, pus is visible to the naked eye under the arachnoid. 
This membrane loses its transparency and begins to appear 
cloudy, the cloudiness being most apparent along the course 
of the vessels from which the exudation undoubtedly takes 
place. 

The pus is found both in the meshes of the pia, and un- 
der the pia, between it and the cortex.* The fibrinous puru- 
lent layer will be found adherent to the pia, and can usually 
be removed together with this membrane. These purulent 
layers can be drawn out of the fissures, leaving discolored 
tissue underneath. This purulent exudation covers not 
only the fissures of the convexity, but extends with equal 
frequency over the base of the brain, and in those very 
spaces in which we are accustomed to look for tubercular 
deposits. In addition to the exudation over the brain 
proper, an equally thick layer can often be found over the 
greater part of the spinal cord, holding the same relations to 
the spinal meninges that it does to the cerebral coverings. 
The blood is apt to be clotted in the large veins and sinuses, 
and such clots may be of a purulent character. The ventricu- 
lar fluid is, as a rule, increased, and in the more violent forms 
may contain small floccules of fibrin or fibrinous pus. 

In addition we may find hypostatic pneumonia, or varying degrees of 
bronchitis and atelectasis ; all the serous membranes may be in a condition 
of inflammation. The spleen is almost invariably enlarged, while the other 
abdominal viscera are found in a condition of decided hypersemia ; the kid- 
neys may be in a condition of acute congestion, and, according to Welch, an 
acute diffuse nephritis is occasionally present. 

* An excellent colored plate of a purulent meningitis may be found in Holt's text- 
book, page 752. 



4IO THE NERVOUS DISEASES OF CHILDREN, 

Differential Diagnosis. — The only point to be con- 
sidered in this respect is the differentiation of this form 
from other forms of meningitis. This differentiation should 
not depend solely upon the bacteriologic findings, but note 
that the disease advances much more rapidly than do the 
tubercular or other types. The mental symptoms are, above 
all things, developed much more actively and vehemently, 
in keeping with the early exudation of pus over the surface 
of the brain. The temperature is higher in the earliest 
period of the disease. Cranial nerve palsies are often want- 
ing. The occurrence of similar disease in the city, the con- 
dition of the environment of the patient, and the exclusion of 
those facts which tend to prove the presence of tubercular 
disease will help to make the diagnosis of the epidemic form 
much more certain. 

Prognosis. — The disease is unquestionably one of the 
most fatal diseases of childhood, and if it does not prove 
fatal the condition of the survivor is often so distressing 
that death would have been preferable. About one-half of 
the patients make a fair recovery. The duration of the 
coma is as reliable a sign as any in giving a prognosis. 
Cases in which the coma is rapidly developed and does not 
show any sign of receding within the first week or two are 
almost certain to end fatally. If the coma has been slow to 
develop, the inference is justified that the process is a less 
intense one, and there is in so far a hope of recovery. But 
even in these cases if the coma which has once been devel- 
oped remains stationary for a week or more, the chances of 
recovery lessen with almost every hour that the coma con- 
tinues. In spite of all rules that may be laid down children 
whom we have reason to expect to recover take a turn for 
the worse, and not a few of those who have been given 
up by the most careful and experienced physicians make 
good recoveries ; but in others, in whom the disease drags 
along, the final issue is simply deferred, and death may 
result from exhaustion, as in one of my own cases, as late 
as four months after the onset of the disease. 

Treatment. — The treatment of this special form of 
meningitis can differ in no respect from that noted in con- 
nection with the discussion of the tubercular and simple 



MENINGITIS. 41 1 

forms of meningitis. Although the disease has not been 
proved to be contagious, the patient should be strictly 
isolated. 

In the epidemic of 1904, Manges, following the recommendation of Seager, 
who reported upon an epidemic in Lisbon, advocated the use of a three 
to nine c. c. injection of a one per cent. Lysol solution into the spinal canal 
after the withdrawal of cerebro-spinal fluid. Three cases, treated in this 
fashion (one of them a streptococcus infection) recovered. In the hands 
of others this treatment has failed. During the present epidemic, the sug- 
gestion has been made to use the Diphtheria Antitoxin in the treatment of 
epidemic cerebro-spinal meningitis. If such treatment prove successful, the 
Bacteriologists will have to revise their fundamental doctrines and beliefs. 

MENINGITIS DUE TO OTHER CAUSES.* 

I. Meningitis due to traumatism has been mentioned in 
connection with acute meningitis ; it is much rarer in chil- 
dren than in adults, for reasons which it is hardly necessary 
to explain; though the injuries which children receive may 
be relatively slight, meningeal disturbances follow upon 
them in some instances. A fall from a chair, a fall down 
the stairs, or a blow dealt upon the head of the child, to 
which little heed is paid at the time, may be the starting- 
point of the meningitis. At times the same slight traumatic 
factor is the cause of an epilepsy developing after six 
months, or even after a year, or still later (see chapter on 
Epilepsy). In these children a local meningitis or meningo- 
encephalitis may be considered to be the actual lesion re- 
sponsible for the epilepsy. With such cases we have no 
concern at present. We must take into account, however, 
those patients in whom some traumatism to the skull is fol- 
lowed by the symptoms characteristic of meningitis. The 
inflammatory process may involve both the dura and the 
pia, and may be either serous or purulent. 

In these days of cranial surgery, another form of (purulent) meningitis is 
to be observed in children and in adults after operative procedures for the re- 
lief of epilepsy or other brain diseases. The aseptic principles of modern sur- 
gery are calculated to prevent such complications. It is exceptional to see 
this condition in cases in which a simple trephining operation has been done, 
but if the dura has been opened the danger of meningitis is greater. 

* For " Meningitis serosa " see page 421. 



412 THE NERVOUS DISEASES OF CHILDREN. 

The prognosis of a meningitis developing after opera- 
tion is, on the whole, excessively grave. The only advice 
to be given is that as soon as the temperature rises, and as 
soon as the first symptoms of a suppurative meningitis set 
in, the wound should be opened at once, and an effort made 
to treat the condition according to the best surgical prin- 
ciples. The same applies to those cases of meningitis which 
follow upon external injuries, if the meningeal symptoms 
can be clearly traced to the preceding injury. A careful 
examination of the skull should be made in order to deter- 
mine, if possible, where the injury has been inflicted. If 
the slightest depression can be felt, trephining should be 
done over this region, and if no changes in the skull can be 
made out, but the symptoms point to an incipient meningitis, 
any abrasion of the scalp, or any extravasation of blood un- 
der the scalp, should be a sufficient guide as to the site of 
the operation. 1 am firmly convinced that much good can 
be done by proper and timely surgical interference in these 
cases, and little harm will result even if the operation should 
prove that no tangible local injury has been done to the 
brain or skull. To be sure the operation should not be un- 
dertaken if the child's general condition is such that it will 
not bear the shock of the operation. In such circumstances 
the calm judgment of an experienced surgeon or physician 
will be of great value, but it should be distinctly stated that 
coma, however profound, or recurrent convulsions, do not 
constitute a contra-indication to operative interference.* 

II. Meningitis in a purulent form may result from dis- 
ease of the ear. The aurists are well aware of the danger 
lurking in every form of disease of the mastoid and of the 
inner or middle ear ; for if the pus that is formed in any one 
of these regions is not discharged outward, the danger of its 
causing a purulent meningitis by a direct discharge inward, 
or through caries of the petrous bone, is much to be feared. 
Chronic ear trouble, so slight that little attention is paid 
to it, may persist for years before causing a purulent raen- 

* Too much valuable time is wasted with the administration of drugs that may ease 
the physician's conscience but do the patient little or no good. If the symptoms point 
to an increase of intracranial pressure, or to an extension of the morbid process, an 
exploratory trephining should be attempted. 



MENINGITIS. 413 

ingitis.* Since aural surgeons have become accustomed 
to operate promptly upon the appearance of symptoms 
pointing to suppuration in the mastoid process, or in the 
other bony parts of the ear, these forms of purulent menin- 
gitis from ear disease are far less frequent than they were 
formerly. The symptoms which point to an incipient 
meningitis can be distinguished in many cases, though not 
in all, from those due to the presence of pus within the ear 
structures alone. In addition to the intense pain, the pres- 
ence of pus in the ear may cause giddiness, intense vertigo, 
vomiting, and even slight stupor, but if the headaches be- 
come general and most intense, if the child becomes coma- 
tose, if the pulse is either slowed up or very much acceler- 
ated, if the neck becomes sensitive and rigid, and if a slight 
optic neuritis should set in in one or both eyes, it is certain 
that the pus has passed beyond the limits of the ear and has 
set up a meningitis, or possibly an abscess. Under such 
conditions it is imperative to operate, giving the pus a 
chance to discharge outward, and to lay bare, if necessary, 
the parts of the brain which are most apt to be involved in 
ear disease. It will be advisable at the start thoroughly to 
explore the mastoid, as well as the middle and inner ear, be- 
fore attempting to trephine over the temporal convolutions, 
but if the operations upon the bony parts of the ear do not 
give prompt relief the cranial operation should be done. The 
aural surgeons are opposed to operations in cases of purulent 
meningitis, but as long as the meningitis is strictly localized 
and confined to the parts in immediate juxtaposition to the 
ear, there is every reason to advise an exploratory operation 
to secure an exit for the pus, which, if confined to these 
parts, is bound to cause a general suppurative meningitis. 

If suppurative meningitis is due to disease of the mas- 
toid, the pus is more likely to find its way either to the cere- 
bellum or to the base of the brain. In diseases of the middle 
and inner ear, the meningitis is more apt to be developed 



* The author saw this well illustrated in a lad of fourteen (a patient of Dr. Wiener), 
who since his fourth year had had chronic ear disease ; without special cause this old 
trouble was lighted up and ended in death within one week. The autopsy disclosed a 
wide-spread purulent meningitis from caries of the right petrous bone. The morbid 
condition was most marked over the left temporo-sphenoidal lobe. 



41 4 THR NERVOUS DISEASES OF CHILDREN. 

in the regions over the first and second temporal convolu- 
tions. In endeavoring to locate the proper region for open- 
ing the skull in cases of ear disease it is best to select a point 
that is reached by going one and a quarter inches back of 
the external auditory meatus, and from this point one and a 
quarter inches upward. If the surgeon remembers that a 
line drawn from the outer angle of the orbit horizontally 
across the skull gives the approximate position of the fissure 
of Sylvius, and therefore the upper limits of the temporal 
convolutions, he cannot well fail to open the skull over the 
part of the brain that comes into question in these cases.* 

Meningitis may also be caused by a tumor or an abscess 
in adjacent parts of the brain ; but in such cases the men- 
ingitis is of relatively little importance, and if it gives 
rise to distinct symptoms simply helps to intensify those 
caused by the chief morbid process. Purulent meningitis is 
sometimes due to disease of the nose, to erysipelas, and to 
purulent disease of the eye. If so, the antecedent condi- 
tions will leave little doubt of the cause of the meningitis, 
and the subsequent symptoms will not vary from the men- 
ingitis following upon other causes. 

III. Many of the infectious diseases of children lead to 
meningitis. Among these are measles, scarlatina, small-pox, 
and even rheumatism and influenza, but, above all, typhoid 
fever and pneumonia. 

Meningitis has been found present in the autopsies upon 
persons dead from typhoid fever, but is by no means pres- 
ent in all those forms of typhoid fever in which at the begin- 
ning or in the later stage of the disease delirium and coma 
are developed in connection with the fever. If a child have 
typhoid, the diagnosis of additional meningitis should not 
be made unless there are very positive symptoms, such as 
decided rigidity of the neck, hyperesthesia of part or of the 
entire body, and cranial-nerve palsies. 

Brain symptoms are not infrequent complications in 
acute pneumonia, and much attention has been directed to 
these cerebral complications by Holt and others ; but menin- 
geal disease unquestionably occurs in a number of instances, 
and we can the more readily understand the connection 

* See chapter on " Abscess." 



MENINGITIS. 415 

between the two diseases sinc'e Fraenkel's pneumococcus 
has been found in the inflamed lung, and in the meningeal 
exudate. The same coccus is also found at times in the epi- 
demic purulent form of meningitis. 

IV. Among the many curious and surprising sequelas 
of influenza which have been described, none has been more 
striking than the occasional occurrence of cerebral and cere- 
brospinal meningitis during or immediately after the acute 
stage of the disease. For the present the evidence is in favor 
of an encephalitis, rather than a meningitis, as the cause of 
this special form of disease. (See p. 416.) 

Inflammation of the meninges also follows upon general 
septicemic processes, but this special causation is much 
more common in the adult than in the child. The typical 
septicemic meningitis is that form which we encounter in 
connection with the puerperal state in women, and from 
which I have seen one most remarkable recovery. There is 
no form of septicemic meningitis in children quite as typical 
as this puerperal meningitis, unless it be that which occasion- 
ally occurs with septic bone disease or with ulcerative endo- 
carditis. 

CHRONIC BASILAR MENINGITIS. 

A special form of meningitis occurring in children has been described by 
Gee, Barlow, Still, and others. Its only claim to recognition is that it may 
run a protracted course varying from months to a year or more, that it affects 
very young children, that the inflammation is most marked in the posterior 
fossa of the base — and that for a long time cervical opisthotonus is the chief 
symptom. Since Still has shown that the diplococcus intracellularis was 
found in seven of eight cases, and since these cases have been observed dur- 
ing epidemics of cerebro-spinal meningitis (Koplik), there is no reason to re- 
gard chronic basilar meningitis as a distinct clinical entity. The designation 
" posterior basic meningitis " is misleading ; tuberculous forms are " posterior 
basic " often enough, as well as anterior basic, or middle basic. Such terms 
produce confusion. 

In the cases so classified, lymph may glue together the medulla and the cere- 
bellum, the openings from the fourth ventricle may be closed, and the ventricles 
distended. The writers advocating this form claim that the symptoms are: 
Gradual or sudden onset, convulsions, slight or no fever, opisthotonus, general 
muscular rigidity, mental dulness and irritability, rarely coma. We agree 
with Holt that some forms of " chronic basilar meningitis " in children may be 
syphilitic. As for treatment, we would urge lumbar puncture, to be repeated 
carefully a number of times, and the administration of iodides, about ten or 
fifteen grains per day, or more — according to the age of the child. 



41 6 THE NERVOUS DISEASES OF CHILDREN. 

ACUTE HEMORRHAGIC ENCEPHALITIS. 

We cannot close the discussion of meningitis without reference to acute 
encephalitis, which is frequently associated with inflammation of the pia, but 
is also at times developed independently of any meningeal trouble. In men- 
ingitis due to traumatism, or to acute and chronic intoxication, some enceph- 
alitis occurs by simple extension of the inflammation. Striimpell, Fiirbringer, 
Leichtenstern, Oppenheim, and others have endeavored to establish a form of 
acute hemorrhagic encephalitis which is developed commonly after some acute 
infectious disease, particularly after influenza. 

Simple acute encephalitis can be recognized by the following signs : The 
disease is very apt to attack children before the age of puberty. For a few 
days previous to the full development of the disease the child complains of 
headache, of dizziness, and is irritable or depressed ; drowsiness, gradually 
developing into coma, soon sets in. Rigors and slight elevations of temper- 
ature point to the development of an acute infectious trouble. The loss of 
consciousness need not be complete, and distinct remissions occur during the 
first week of the disease. Hours of stupor are followed by a condition of 
wakefulness and restlessness. The pupillary reflexes may remain normal or 
may be sluggish ; the deep and superficial reflexes are not altered. A rigid- 
ity of the neck and slight opisthotonos are early symptoms. Paralysis, in 
the form of monoplegia or a hemiplegia, is observed at an early day, and may 
or may not be associated with aphasia.* Ocular and other cranial nerve pal- 
sies are developed, and if they set in in an acute fashion the condition may 
remind one very easily of a basilar meningitis, with the exception of the in- 
complete loss of consciousness. Respiration may become irregular, and the 
cardiac action is either accelerated, irregular, or diminished. Optic neuritis 
has been observed in several such cases. The symptoms will vary very much 
according to the seat of the disease. Loss of consciousness, convulsions, and 
palsies will be more frequent in encephalitis of the convexity, whereas cranial 
nerve palsies, dizziness, difficulties in deglutition and in articulation may be 
expected if the encephalitis is developed chiefly in the basilar structures. 

The cause of the disease will vary also according to the intensity of the 
encephalitis. In some of the cases the coma deepens, the patient may linger 
on for two or three weeks, but finally succumbs to the disease, the entire 
course reminding one very much of the pyaemic forms of ncephalitis. In 
other patients, after the disease has lasted for a week or two, prolonged 
remissions and complete recoveries may set in. 

As an instance of encephalitis following influenza, I wish to refer to the 
following case, although the possibility of a meningitis being associated with 
the encephalitis cannot be denied. The patient and several other members 
of the family had passed through the usual attacks of influenza four days 
previous to my first visit. The girl, eighteen years of age, had been badly 
frightened by the falling of a picture. That same evening she began to 
vomit and complained of severe headaches. The family physician, who ex- 

* On the relation ot the acute infantile cerebral palsies acute encephalitis, cf. Chap- 
ter XXV 



MENINGITIS. 4 1 7 

amined her, found a temperature of 103 F., and noticed at once a slight re- 
traction of the neck. For the next few days the temperature varied between 
101 and 103 F. The rigidity of the neck became more pronounced, but 
the pulse and respiration were not sensibly affected at any time, the former 
being at the rate of 90 and the respirations varying between 16 and 20 a 
minute. On the fourth day of the disease there was a very marked rigidity of 
the neck and great painfulness over the cervical and dorsal spine ; excessive 
muscular tenderness in the lower extremities, such as is frequently found in 
cases of simple influenza. The deep reflexes were lively, but not excessively 
exaggerated. The patient was drowsy ; if left to herself would sleep, but 
when aroused answered all questions intelligently. She presented in addition 
a marked paralysis of the rectus externus in both eyes. There was loss of light 
reflexes and sluggishness of contraction during accommodation in both eyes, 
but there were no other third-nerve symptoms. The papillae were somewhat 
swollen. These symptoms continued, with slight changes, for two weeks ; 
then she began to improve slowly ; four weeks after the onset of the first 
symptoms the patient was entirely well. 

These cases surely bear a strong resemblance to the ordinary forms of 
meningitis, and were it not for the evidence furnished by Fiirbringer, Leyden, 
Eisenlohr, and Koenigsdorf that encephalitis is the actual condition, the 
diagnosis of a meningitis could be defended quite as readily. 

Morbid Anatomy. — As far as can be determined at the present time, 
the morbid process is a simple hemorrhagic encephalitis. The inflammatory 
areas are small and strictly circumscribed, and may be developed in symmet- 
rical parts of the brain. On superficial examination the brain tissue appears 
hyperasmic and dotted with small red points ; the areas of inflammation are a 
little softer to the touch than normal tissue. On microscopical examination 
the smaller vessels are found dilated to their utmost capacity, filled with 
blood, some of which has evidently been exuded into the neighboring tissue. 
Leucocytes, granular cells, and a proliferation of the cells of the neuroglia 
complete the microscopical picture. There may be slight destruction of the 
nervous elements, but this will depend upon the intensity of the process. 
The entire encephalitis is, no doubt, of microbic origin. Southard and Keene 
have very recently shown that it may be due to the staphylococcus pyogenes 
aureus. 

The prognosis will naturally vary according to the intensity of the symp- 
toms . A fatal issue is apt to follow in those cases in which the symptoms, 
coming on with great violence, point to an intensely septic process. If the 
condition of coma is reached by slow stages and remissions are observed 
within the first week or ten days, a favorable turn may be expected. 

The treatment will consist simply in absolute quiet, the application of cold 
to the head and nape of the neck, and in the administration of purgatives 
(calomel is by far the best). 

Polio-encephalitis Superior. — A disease due to the inflammation 
of the gray matter of the floor of the fourth ventricle and of the aqueduct of 
Sylvius, had been described by Wernicke, Thomsen. and others. This dis- 
ease has been observed in the adult, and is remarkably frequent in persons 



41 8 THE NERVOUS DISEASES OF CHILDREN. 

presenting the symptoms of chronic alcoholism. This special form would 
naturally be a rarity in children ; the symptoms are, however, not unlike that 
form of encephalitis which has been described in connection with influenza. 
It is of particular interest, also, because a similar condition is at times asso- 
ciated with polio- myelitis. We have often referred to the close relation be- 
tween the gray matter in the spinal cord and the gray matter which harbors 
the nuclei of the cranial nerves. We can readily understand why the same 
morbid process should at times affect the cerebral, and at other times the 
spinal portion of this central gray matter. According to the distribution of 




Fig. 103.— Case of Unilateral Nuclear Palsy. (Wiener.) Hypoglossal nucleus, left 
side. Photomicrograph from a section stained after Pal, showing .some degenerated 
ganglion cells. 

the inflammation in the region of the ocular nerve nuclei, or in the vicinity of 
the nuclei of the tenth and twelfth nerves, we distinguish between polio- 
encephalitis superior and inferior. The disease in which ocular nerve symp- 
toms (ophthalmoplegia, partial or complete) have been associated with polio- 
myelitis, is termed polio-encephalomyelitis. This form has been described 
by Rosenthal, Seeligmiiller, Guinon, myself, and others. But these diseases 
are, on the whole, extremely rare, and do not, as a rule, begin until childhood 
is past. The cases also take a more or less subacute course. 

Bulbar Palsies.*— Diseases of the pons and medulla are very rare in 
children ; but the above discussion of nuclear diseases leads to the mention 

'The author had some hesitation in discussing these diseases in this chapter ; but 
they are too rare to warrant discussion in a separate chapter, and it did not seem 
desirable to add them to the sections on congenital nuclear palsies. 



MENINGITIS. 419 

of progressive bulbar palsy. This disease in the adult bears a close clinical 
and anatomical relation to progressive amyotrophy and to amyotrophic lateral 
sclerosis. With the chronic diseases of adults we are not concerned, but a 
short allusion may be made to progressive bulbar palsy of earlier years, 
which is a rare disease, but, like the adult form, is due to a primary degen- 
eration of the nuclei of the lower cranial nerves.* A case of this descrip- 
tion, from my clinic, was carefully studied and described by Dr. Wiener. 

The symptoms of unilateral bulbar palsy were discovered quite acciden- 
tally in a young man who had been under treatment by his physician for tu- 
bercular glands and pharyngitis. He presented : 1. A very marked deviation 
of the tongue to the right when protruded. 2. Distinct atrophy of the me- 
dian portion of the right half of the tongue. 3. The faradaic response of 




Fig. 104. — Same Case as Fig. 103. Hypoglossal nucleus, right side, showing absence 
and extreme degeneration of ganglion cells. 

the right half of tongue was much diminished ; the contractions were slug- 
gish, there was increased galvanic excitability of the right side (K. C. C.< 
A. C. C.) ; the contractions were slow and wave-like. 4. Taste and tactile sen- 
sations were normal. 5. Deviation of the soft palate and of the uvula to the 
left side. On phonation the paralysis of the right side became more marked. 
6. Great difficulty in deglutition. 7. Dysphonia due to disease of the right 
recurrent laryngeal nerve. 

Other cranial nerve functions were normal. All reflexes normal. 

A gradual progression of all the symptoms led to a fatal issue from respira- 
tory failure. Examination of the brain revealed a distinct degeneration of the 
hypoglossal and vago-accessorius nuclei of the right side, with degeneration 
of the respiratory column. In the hypoglossal nucleus, the ganglion cells 
were greatly changed (atrophied, shrunken, and granular). The ground 

* No special reference is here made to the acute and the pseudo-bulbar palsies, as 
both these conditions are very rare in children An acute polioencephalitis inferior in 
young subjects, giving rise to bulbar symptoms, has been described. 



420 THE NERVOUS DISEASES OF CHILDREN. 

substance did not stain as readily in the right as in the left, and was less 
compact ; the left hypoglossal nucleus was also slightly diseased (lower and 
outer portions). (See Figs. 103, 104.) 

The symptoms of bulbar palsy (paralysis and atrophy of the muscles of 
the palate, the tongue, the pharynx, and the larynx) were distinctly present. 
In some subjects there is additional paralysis of the lips and of the muscles 
of mastication, and also of the upper divisions of the facial distribution. 
The difficulties of deglutition and the dysarthria were characteristic ; all these 
symptoms justify the clinical designation of glosso-labio-pharyngeal paral- 
ysis. The unilateral character of the symptoms is worthy of note, but does 
not remove the case here recorded from the category of subacute or chronic 
bulbar palsies. 

The disease which involves the nuclei of the motor cranial nerves is occa- 
sionally associated with spastic symptoms, with increase of the deep reflexes, 
and with atrophy in the upper extremities. The clinical symptoms thus bear 
the closest resemblance to those ofamyotrophic lateral scleros is and the 
latter disease may well represent an extension of the bulbar process into the 
spinal cord ; the spinal symptoms of amyotrophic lateral sclerosis may pre- 
cede the bulbar symptoms for a long period of time. All these forms repre- 
sent a disease of both divisions of the motor tract. 

C. H. Brown has described an interesting case of amyotrophic lateral 
sclerosis with bulbar symptoms which exhibited the first signs at the age of 
twelve years or earlier. All the facial muscles were involved ; the boy could 
not laugh or swallow easily; speech was nasal; there was atrophy of the 
sterno-cleido-mastoid of the left side, and to a lesser extent of the right. 
Other neck muscles weak. Considerable atrophy around the shoulder gir- 
dle, of all of the arm muscles, and of the interossei and the thenar group, 
fibrillary twitchings and increased reflexes completed the series of symptoms. 
Progressive bulbar paralysis in children has also been described by Hoff- 
man, Remak, and Londe. Eisenlohr has described a bulbar form of infantile 
spinal paralysis. 

Charcot, Londe, Brissaud, Marie, Hoffman have described an infantile, 
hereditary or family form of progressive bulbar paralysis. The characteristic 
signs, in addition to the bulbar symptoms, were the presence of stigmata of 
degeneration, the involvement of the upper facial branches, the occurrence of 
ptosis and of a partial or complete ophthalmoplegia. 

Oppenheim, Peritz, Bouchaud, Ganghofner have described an infantile 
for?n of pseudobulbar paralysis. This complex of symptoms is developed 
in association with the infantile cerebral palsies. Glosso-labio laryngeal 
symptoms may be combined with spastic-athetoid and diplegic symptoms. 
Oppenheim and Bouchaud have shown this form to be due to defective devel- 
opment (porencephaly) of the lower portion of the central convolutions. 



CHAPTER XXIV. 

HYDROCEPHALUS. 

By hydrocephalus we mean an excessive accumulation 
of serous fluid within the cranium, either in the subdural 
spaces or in the ventricles. The former is termed exter- 
nal hydrocephalus ; the latter, internal hydrocephalus. 
It is convenient also to divide the cases into acute and 
chronic forms, and to subdivide the latter into congenital 
and acquired hydrocephalus. As the accumulation of fluid 
often follows upon other diseases, we distinguish a form of 
secondary hydrocephalus ; the term primary hydrocephalus 
remains restricted to the few cases in which the accumu- 
lation of fluid appears to be the sole morbid condition. 
Chronic hydrocephalus is by far the more important, for 
what might be said of truly acute hydrocephalus has already 
been said in connection with the primary forms of meningi- 
tis ; but a few remarks will be in order, at this juncture, in 
relation to the subject of acute hydrocephalus, neglecting 
for a time the hydrocephalic condition which accompanies 
every form of meningitis, and particularly the tubercular 
variety. 

Acute Hydrocephalus. — Quincke, in an able article 
on meningitis serosa states, that the term acute hydrocepha- 
lus should be made to cover the condition in which there is 
a purely idiopathic serous meningeal inflammation. In the 
cases which he describes, and of the existence of which there 
is no doubt, the symptoms point to a very constant increase 
of intracranial fluid in the subpial spaces and within the 
ventricles. Acute hydrocephalus is characterized generally 
by very sudden onset ; occasionally the symptoms come 
on in insidious fashion. Fever may be entirely wanting, or, 
if present, rarely exceeds 103 F. ; headache, rigidity of the 



422 THE NERVOUS DISEASES OF CHILDREN. 

neck, nausea, vomiting, stupor, coma, delirium, are the symp- 
toms present in this disease, and they are the same which 
we have insisted upon so frequently in connection with the 
various forms of meningitis. The pupils react sluggishly, 
and are often unequal ; optic neuritis is present ; convul- 
sions may occur, but are rare, and palsies of a cerebral 
order may be present, but are rarely either severe or lasting. 
Paralysis of the external rectus muscle is as frequent as any, 
and this can readily be explained by increased intracranial 
pressure. In milder cases the symptoms soon recede, and 
absolute recovery may take place within a few weeks. In 
the more serious forms the symptoms may closely resemble 
those of brain tumor. The disturbance of function due to 
increased intracranial pressure may bring about a fatal issue. 
Some of these types of meningitis serosa are no doubt idio- 
pathic, others may represent an acute exacerbation of a 
chronic hydrocephalus. These diseases occur rarely before 
the first year of life, but are most frequent between the age of 
one and five years. They may occur up to the age of thirty 
years, and even later. 

Meningitis serosa may be due either to traumatism or 
to acute febrile disease, such as typhoid and pneumonia ; in 
the adult the excessive use of alcohol, and the pregnant 
state may be responsible for the development of the disease. 
It is probable that this serous form of meningitis is not of 
microbic origin. A distinction should be made in fact be- 
tween these cases and those in which some micrococci are 
known to be the definite cause. 

Pathology. — Acute hydrocephalus may be due to a 
number of different causes. First, to venous stasis, such as 
occurs in connection with severe heart disease. Secondly, 
venous stasis in the larger veins at the base, due to neoplasm 
in the posterior cranial fossa, may also be the direct cause of 
acute dropsy of the brain. Thirdly, stasis in the lymphatic 
vessels, most frequently due to tumors in the posterior fossa, 
causing compression of the subarachnoid lymph spaces and 
of the aqueduct of Sylvius, are a sufficient cause of acute 
hydrocephalus. Fourth, any acute inflammatory process 
of the brain or of the meninges may lead to this form of 
meningitis. The exact symptomatology of an affection of 



HYDROCEPHALUS. 4 2 3 

this nature occurring in a young person may be illustrated 
by the following history taken from Quincke : 

A boy, nine years old, who was said always to have had a large cranium, 
was struck upon the back of the head. He was unconscious for a few min- 
utes, and after the accident complained of pains in the occiput, which were 
generally slight, but occasionally much increased. With increasing headaches 
the boy grew pale, vomited, showed slight rigidity of- the neck, and retraction 
of the head. The horizontal circumference of the head six months after the 
accident was 54^ ctm. No pain on percussion of the head, but pressure upon 
the upper cervical spinous processes was painful. Vision much diminished. 
The right eye had fair perception of light ; with the left the boy was able to 
count fingers. The pupils moderately dilated, reacting sluggishly; both 
papillae were atrophic. All movements were perfect. Serous fluid was with- 
drawn in considerable quantity on two different occasions by Quincke through 
a puncture in the lumbar region. Upon this there was improvement in the 
headaches, but sight remained permanently impaired. 

Chronic Hydrocephalus. — From this category of 
cases we may at once exclude those in which the increase of 
intracranial fluid is of a simple compensatory nature. Such 
an increase is found in many instances of congenital imper- 
fect development of the brain. A skull of averag'e dimen- 
sions sometimes harbors a very small brain, the cranial cav- 
ity being filled by an increased amount of fluid. The fluid 
causes a bulging of all the cranial bones. The accumulation 
of fluid during the intra-uterine period may increase the size 
of the head to such an extent as to make it a serious obstacle 
to normal delivery. 

Deficient brain development is at times limited to one- 
half of the brain. An entire hemisphere may be trans- 
formed into a large serous sac, while the other half presents 
tolerably normal appearance. An entire hemisphere may 
consist of nothing more than a superficial layer of gray 
matter bordering upon a huge cyst which communicates 
with the ventricles. I have had opportunity to be present 
at an operation upon such a brain in which a cyst was di- 
agnosticated, but the size of the cyst not suspected. On 
opening this cyst the surgeon's probe could be passed many 
inches forward and backward, showing that the greater 
part of the hemisphere consisted of this cystic mass. The 
boy died about two weeks after the operation, when it was 



424 THE NERVOUS DISEASES OF CHILDREN. 

•found that the entire hemisphere consisted of nothing else 
than a small layer of gray matter bordering a huge cystic 
cavity. But all these forms, however interesting they may 
be, have comparatively little value, and are far less frequent 
than those in which there is a condition of congenital hy- 
drocephalus. 

Congenital Hydrocephalus. — For reasons which are 
not well known an excessive accumulation of fluid may occur 
within the ventricular cavities during the intra-uterine pe- 
riod. The condition is found in children of absolutely 
healthy parents after normal pregnancies, and in families in 
which all other children have been entirely healthy. In 
some families, however, several children are born afflicted 
with this same trouble ; one or the other may survive while 
the majority are either still-born or die very soon after 
birth. Syphilis of the parents, alcoholism, tuberculosis in 
the father or mother, emotional excitement of the mother 
during pregnancy, injuries to the mother during this same 
state, all these factors have been held responsible for the 
condition, and no doubt are operative in some of these 
patients, while in the majority the actual cause remains un- 
known. If hydrocephalus is present at the time of birth 
the amount of fluid present may go on increasing within 
the first few days, or as long as the child lives. 

In this connection the observations made upon an infant whom I had 
occasion to examine in 1893 may be of some interest. When I saw it 
for the first time it was four weeks old. The mother had given birth to one 
other girl, three years previously, that had remained healthy and showed no 
signs of internal hydrocephalus. The father was a man of remarkably vig- 
orous health. During this second pregnancy the mother menstruated regu- 
larly. On account of the size of the head labor was extremely difficult, but 
the child was uninjured when born. The measurements which were taken 
at three different occasions will show the rapid increase of the fluid, as indi- 
cated by the rapid increase in the head measurements : 

Aug. 6. Aug. 8. Aug. 10. 
Inches. Inches. Inches. 

Horizontal circumference 20^ 21^ 22 

Naso-occipital measurement 13^2 I2>^ 14K 

Bin-auricular measurement 12^ 13^ 13 

On the 9th, between the second and third measurements, a large amount 
of fluid was withdrawn by tapping the fontanelles, but it will be noticed that 



HYDR O CEP HA L US. 42 5 

the fluid was rapidly replaced. The child lingered on for another week and 
then died, after having become greatly emaciated. More than a week before 
its death it could not be fed by the mouth or per rectum. 

But all cases of congenital hydrocephalus do not take 
such a serious turn, and in some of them the accumulation 
of fluid ceases and a tolerably fair and even normal mental 
development may ensue. The protruding occipital bones 
clearly visible on so many bald heads point to a moderate 
amount of internal hydrocephalus in the earlier years of 
life. Some of these may have been acquired in the earlier 
period of life and are not necessarily congenital in origin. 
The fluid accumulates most readily within the lateral ven- 
tricles, both in the anterior and in the posterior horns. The 
aqueduct of Sylvius may be distended into a funnel shape 
by the increased fluid, but the fourth ventricle, as a rule, 
suffers very little. The brain is compressed by the ac- 
cumulation of fluid, and is often so flattened and thinned 
that its thickness is not greater than that of ordinary thick 
paper. The white matter seems to yield more readily than 
the gray, and I have been struck, in a number of different 
brains, by the fact that the function of the parts remained 
normal in spite of the extreme thinness of the cerebral tissue. 
Thus in one instance in which both occipital lobes were re- 
duced to the thickness of paper, sight had remained entirely 
normal up to the age of two years, at which time the patient 
died. The membranes are, as a rule, normal, or else show 
only very slight thinning, and on microscopical examination 
only the slightest traces of acute inflammation or none at 
all. The choroid plexuses are generally thickened, as is 
also the limiting membrane of the ventricles. 

The fontanelles bulge and pulsate distinctly. The con- 
figuration of the skull is modified by the excess of internal 
hydrocephalic fluid. If the hydrocephalus is congenital, or 
if it has occurred in very early life, the bones yield to the 
increased pressure within and the skull becomes distended 
in all directions. In keeping with the greater accumulation 
of fluid in the anterior and posterior horns of the ventricles 
and the protrusion of corresponding parts of the brain, the 
greatest amount of bulging is observed in the frontal and oc- 
cipital bones respectively. The effect of the excessive ac- 



426 THE NERVOUS DISEASES OF CHILDREN. 

cumulation of fluid in the posterior horns of the ventricles 
results in a great bulging of the occipital bones, with marked 
increase in the transverse occipital diameter of the skull. 
If the child survives, the congenital internal hydrocephalus 
gives rise to a number of symptoms pointing to deficient 
cerebral development. On account of the size and weight 
of the head the child is rarely able to carry the head unsup- 
ported. As it grows older it may be able to sit in a chair, 
but the head generally inclines to one side or the other, or 
forward, in which case it may rest upon the chest. Defec- 
tive mental development is present in the majority of these 
cases, ranging between total idiocy and varying degrees of 
imbecility ; some of the children acquiring a slight use of 
language and others learning to utter only a few words. 
Wyss states that of forty-five hydrocephalic children only 
five were able to attend school. Cerebral palsies with typi- 
cal spastic contractures are quite frequent, and convulsive 
seizures amounting to a chronic epilepsy often add to the 
burdens of the child, and to the distress of the mother who 
is to care for it. In some cases there is total blindness, due 
to optic-nerve compression, but the most surprising feature 
of all is the remarkable preservation of the brain functions. 

Acquired Internal Hydrocephalus.— Chronic internal hydro- 
cephalus, not of congenital origin, is a rare disease, and it is at least in rare 
instances only that the diagnosis of such a form of internal hydrocephalus 
can be made, and if made, the cause which gives rise to it is so far more im- 
portant that the internal hydrocephalus is a mere incident and does not de- 
serve much notice. This acquired internal hydrocephalus may be of pri- 
mary origin or it may be due to some mechanical obstruction causing venous 
stasis. The primary form may be a symptom of severe general anaemia, and 
under such conditions it is a counterpart of serous transudation occurring in 
various cavities of the body. It may also occur in children who have rick- 
ets, or in those whose skull is sufficiently yielding to permit enlargement of 
the cranial cavity. The increase of fluid is to a certain extent compensatory 
in such cases. 

Internal hydrocephalus due to mechanical obstruction of the veins of 
Galen, was first described by Whytt. The commonest form of obstruction 
is that due to tumor in the posterior fossa. Through such obstructions the 
foramen of Majendie may become occluded and dilatation of the third ventricle 
will result ; in the case of occlusion of the foramen of Monro, a distention 
will take place in the lateral ventricle, but increased secretion of serous 
fluid may be sufficient to cause distention of all the ventricles without any 



HYDR CEP HA LUS. 427 

obstruction whatever. On the other hand, a meningitis may be sufficient to 
occlude these small openings connecting the ventricles with one another, 
and may. thus become the actual cause of an internal hydrocephalus. This 
special cause of internal hydrocephalus is of interest only in those compara- 
tively few cases which survive a severe attack of meningitis. A slight in- 
crease of intracranial fluid is present in almost every case of intracranial 
growth, and is responsible for many of the general cerebral symptoms ac- 
companying such diseases ; but as we said above, the recognition of tumor 
is so much more important than the diagnosis of an accompanying hydro- 
cephalus that the latter loses very much in importance. 

The symptomatology of the acquired form of internal hydrocephalus dif- 
fers in no respect from that of other chronic forms except that the symptoms 
develop with some suddenness ; vertigo, dizziness, stupor, coma, convulsions, 
contractures, and amblyopia, preceded possibly by a condition of hemianopsia, 
are the symptoms which should be looked for in these patients. It stands to 
reason that if the internal hydrocephalus originates at the time when the bones 
are still yielding, the injury to the brain and the symptoms resulting therefrom 
will be far less marked than they will be if it occurs after complete ossification 
of all the sutures and bones. After the sutures have been closed the increas- 
ing hydrocephalus may force them open again. I have not observed this in 
any of the primary forms of hydrocephalus, but have seen it occur in a child 
of four years, who died of cerebellar tumor with enormous internal and ex- 
ternal hydrocephalus. The acquired internal hydrocephalus runs very much 
the same course as the chronic form, and is very apt to terminate fatally in a 
few weeks or months. Moderate cases may survive for a longer period of time. 

The diagnosis of all forms of internal hydrocephalus de- 
pends entirely upon the association of an enlargement of the 
cranial cavity with symptoms which point to an increase of 
intracranial pressure. The exact form of hydrocephalus, 
whether it be congenital or acquired, can be diagnosticated 
only with reference to the history of the individual. In en- 
deavoring to determine whether the hydrocephalus is in- 
ternal or external, we should remember that the external 
hydrocephalus is more apt to be congenital and to be asso- 
ciated with symptoms of defective mental development ; and 
furthermore, that bulging of the frontal or occipital bones 
only accompanies internal hydrocephalus ; whereas, a gen- 
eral enlargement of the skull in every diameter, with bulg- 
ing fontanelles and sutures forced apart, indicates the pres- 
ence of external hydrocephalus, which is in most cases as- 
sociated with distention of the ventricles as well. But in 
many children the distinction between external and internal 
hydrocephalus is difficult to make, for the internal hydro- 



428 THE NERVOUS DISEASES OF CHILDREN. 

cephalus may have so thinned out the cerebral substance as 
to bring the ventricular fluid very near the surface. 

Further difficulties of differential diagnosis which arise 
occasionally are to distinguish from hydrocephalus the en- 
largement of the skull due to rickets, to syphilis, or to thin- 
ning of the skull resulting from other causes. In the case of 
rickets, the entire history of the disease will give a clew, and 
other symptoms of rickets will be present and will indicate 
the true nature of the enlargement of the head. But both 
rickets and hydrocephalus are not infrequently associated 
with each other. An increase in the size of the skull may 
simulate the condition of hydrocephalus. Such thickening 
is extremely rare, but is met with in the subjects of hered- 
itary syphilis. I have seen this but once, in a boy, aged six 
years, in whom there were other signs of hereditary syph- 
ilis ; yet the skull is never so much enlarged as in the ex- 
treme cases of hydrocephalus, and generally impresses one 
as being unusually hard. 

Prognosis. — The prognosis of hydrocephalus is always 
serious, but depends greatly upon the original cause. Fort- 
unately a goodly number of children with varying degrees 
of hydrocephalus not only live a number of years, but get 
well. The congenital cases are more rapidly fatal than the 
acquired form, and the prospects of a normal mental de- 
velopment are always slight, though remarkable exceptions 
may occur. Measurements of the skull taken at regular 
intervals will enable the physician to gauge the rate of in- 
crease in the accumulation of fluid, and will help him to 
prognosticate the future course of the disease. 

Treatment. — In the milder forms of hydrocephalus, the 
iodides and the preparations of iron will do good service. 
In the severer forms of hydrocephalus the attempt should be 
made to absorb some of the fluid which is present in excess 
by the use of the mercurials and diuretic measures. I can- 
not state that I have seen any favorable results following 
upon this course of treatment, though some temporary re- 
lief has been afforded in a few cases. The treatment is, 
however, a thoroughly rational one. In almost every form 
of acute and increasing hydrocephalus, however little faith 
he may have in the efficacy of the method, the physician or 



HYDR O CEP HA L US. 42 9 

surgeon will be tempted to follow the example of Keen, 
Kocher, Bergmann, Chipault, to tap the ventricles, and thus 
drain away some of the fluid. The fluid is replenished so 
rapidly that very little good can be effected in this way, and 
if it is drained off suddenly the danger of collapse is very 
great. Henschen's statistics have been most discouraging. 
At the present day, lumbar puncture will take the place of 
this procedure, but is, of course, useless in those cases in 
which the communication between the ventricles and the 
cerebro-spinal canal is interrupted. Instead of draining 
away a large quantity at one time, it seems much more 
rational to attempt some method of gradual drainage. 

Every other treatment, including the injections of iodine, the application 
of elastic bands about the head, and of compression of various kinds, is use- 
less. I have often attempted to reduce the skull by applying bandages more 
or less firmly around the head, but the result has in ever}' instance been equally 
unsatisfactory. If the skull is firmly compressed the internal pressure is nat- 
urally increased, and if the compression is not firm enough the bandages will 
do no good. 



CHAPTER XXV. 

INFANTILE CEREBRAL PALSIES. 
(SPASTIC HEMIPLEGIA, DIPLEGIA, PARAPLEGIA.) 

The investigators of recent years have with few ex- 
ceptions endorsed the classification of the cerebral palsies, 
as given in the first edition of this book. We may divide 
these palsies into three groups according to time of onset — 
during the intra-uterine period, during labor, or after birth. 
A clearer understanding of all these disorders will be 
reached by discussing them together, comparing and con- 
trasting the chief features of the one series with those of the 
other groups. 

The subject is not a new one, yet the various forms of cerebral palsies did 
not attract the attention which they deserved until two decades ago. A clear 
account of their clinical symptoms was given by Von Heine in the second 
edition of his book on " Infantile Spinal Palsies " (i860). Heine distin- 
guished between the typical cases of infantile spinal paralysis and those of a 
purely cerebral character. Before his day some of the older French authors 
— Cazauvielh, Breshet, Cruveilhier — had studied the atrophic changes in the 
brains of children who had been afflicted with various forms of palsy. These 
writers were interested in the anomalies of brain structure rather than 
in the clinical features presented by such cases. In 1842 Henoch published 
a dissertation entitled " De Atrophia Cerebri," in which he described the 
brain condition associated with infantile cerebral hemiplegia. The chief ad- 
vances were due, however, to Cotard, a pupil of Charcot, who in 1868 pub- 
lished " A Study on the Partial Atrophy of the Brain," in which he analyzed 
the different morbid processess to which such atrophy might be due. Cotard 
was first also to recognize the importance of traumatic encephalitis as a factor 
in the causation of these conditions. A special study of brain defects was 
made by Heschl, who introduced the term " porencephaly," and by Kundrat, 
who in 1882 published a monograph on this same subject, in which he dis- 
tinguished between congenital and acquired porencephaly, and attempted to 
explain all these conditions by attributing them to a form of anaemic necrosis. 
In 1883 Audry published a collection of one hundred and three cases of this 



INFANTILE CEREBRAL PALSIES. 43 1 

peculiar condition. Lobar sclerosis, a condition to which Cotard had called 
attention, was made the special subject of papers written by Bourneville, 
Richardiere, Jendrassik, and Marie. The last author introduced a decided 
advance in the discussion of the subject by connecting this lobar sclerosis 
with changes in the blood-vessels, and still the character of the initial affec- 
tion remained rather doubtful. Quite apart from all these French and 
German authors, Little, in 1853, had studied carefully the spastic palsies and 
rigidities of children, and showed that they were largely due to protracted 
labor and premature delivery. The conditions due to these two causes have 
since been known, particularly among German and French authors, as " Little's 
Disease." (Cf. page .) 

A fresh impetus was given to the study of all these palsies by the lecture 
of Striimpell, delivered in 1884, who declared that acute infantile spastic 
hemiplegia was due to a primary acute encephalitis affecting the gray matter 
of the cord. He insisted upon an analogy between this acute cerebral and 
the acute spinal palsies, and in order to fortify the resemblance between the 
two sets of disease, proposed the term " polio encephalitis " as the cerebral 
counterpart of " polio-myelitis." The theory advanced by Striimpell was in- 
genious ; there is more clinical than anatomical evidence to support it. We 
can not, even at this day, agree with Leube, who states that " hemiplegia in 
young children should in the first instance be considered to be the result of 
a former acute encephalitis." The truth is that encephalitis is the rarest 
cause of these palsies, and should not be considered at all, unless every other 
morbid state can be safely excluded. But of this later on. 

Strumpell's lecture was the starting-point of the innumerable studies 
which have led to the clearer recognition of the true pathology of these 
disorders* 

The brain palsies of children are far more frequent than 
they are generally supposed to be. In the Hospital for 
Ruptured and Crippled in this city, Dr. Townsend, at my 
suggestion, was kind enough to tabulate the cases of spinal 
and cerebral infantile palsies which presented themselves 
for treatment at that institution for a definite period of time. 
His statistics show that during that period ninety-one 
patients with infantile cerebral palsies, and one hundred and 
forty-two cases of infantile spinal palsies were received for 
treatment. 1 had, during a period of five years, seen over 
three hundred cerebral spastic palsies, but this may be 
due to the fact that many physicians knowing my interest 

* Among the many writings the most prominent are those of Bernhardt, Wallenberg, 
Kast. Moebius, Feer, Freud and Rie, and Oppenheim, in Germany ; of Marie, Cotard, 
Audry, Gibbotteau, in France ; of Ross, Hadden, Gowers, and Ashby, in Fngland ; 
in this country numerous articles have been contributed to this subject by Weir 
Mitchell, Sarah McNutt, Lovett, Osier, Peterson, Sachs, Starr, Spiller, and Dercum. 



432 



THE NERVOUS DISEASES OF CHILDREN. 



in the subject were at special pains to refer such cases to 
me. 

Whatever the time or manner of origin may have been, 
all cases of cerebral palsy in children are so similar in many 
respects that we may attempt to study the symptoms com- 
mon to all. These various forms of cerebral palsies are 
observed most frequently in the 
earlier years of life, from the time 
of birth up to the age of ten years, 
and even later, but by far the great- 
er majority of them occur during 
the first three years of life. The 
paralysis is of the distinctly spas- 
tic order and according to the dis- 
tribution of the palsy we may dis- 
tinguish between a hemiplegia and 
diplegia (double hemiplegia) or 
paraplegia. Monoplegias are rela- 
tively rare. The rigidity of the 
muscles, the contractures resulting 
from the same causes, and increase 
of all the deep reflexes are the con- 
stant accompaniment of these pal- 
sies. Coma and convulsions occur 
in the initial stage of the acute 
forms, and the convulsions at least 
are often repeated during the later 
stages of the disease. The cere- 
bral palsies of children are more 
commonly associated with coma 
and convulsions than are those of 
the adult ; the former are generally 
due to cortical processes, the latter 
to intra-cerebral conditions. The frequent repetition of 
convulsions is equivalent to the establishment of an epi- 
lepsy which may continue throughout life, and the same 
changes which have caused the epilepsy may also be re- 
sponsible for the defective mental development, which may 
range from weak-mindedness to marked imbecility and com- 
plete idiocy. Disturbances of motion, associated, ataxic 




Fig. 105. — Left Hemiplegia. 
Onset in Second Year. Con- 
tractures of arm and fingers. 
(See also Fig. 75.) 



INFANTILE CEREBRAL PALSIES. 433 

athetoid, choreiform, and even cataleptic movements, occur 
quite often in connection with these diseases. All of them 
may be grouped under the general heading of post-para- 
lytic (not necessarily post-hemiplegic) disturbances. Apha- 
sia, so common in apoplectic disorders of the adult, is a 
rarer complication in infantile palsies. 

Among the negative symptoms which are of the greatest 
importance in attempting a differential diagnosis between 
the cerebral spastic and the spinal palsies, we may mention 
the entire absence of changes in the electrical reactions and 
the development of only slight atrophy in association with 
the palsy. Disturbances of sensation are rare, but as these 
are absent in the spinal forms as well, they help little in 
attempting a differential diagnosis between these two im- 
portant series of diseases. 

Let us now take up the symptoms seriatim, and in doing 
so we shall be able to develop the many interesting features 
of infantile cerebral palsies. 



Distribution of Paralysis. — The following analysis of 225 cases is 
based upon the collections made by Peterson and myself : 

Males. Females. Total. 

Right hemiplegia 51 30 81 

Left hemiplegia 40 35 75 

Diplegia 21 18 39 

Paraplegia 22 8 30 

Total 134 91 225 

Monoplegias are so rare that they scarcely enter into the consideration of 
the problem. From the above statistics it will be inferred that right and left 
hemiplegias are more frequent that the bilateral forms of palsy, which is 
equivalent to saying that unilateral cerebral processes are more common than 
double cerebral lesions. There is no difference more striking between the 
adult and the infantile palsies than that implied in the relatively large per- 
centage of cases of cerebral diplegia and paraplegia. In the adult a bilateral 
cerebral lesion is a great rarity, but in children diplegias constitute about 
fifteen per cent, of all the cases, and paraplegia about fourteen per cent. 
This difference between the adult and infantile brain is due first of all to the 
fact that both hemispheres in children are exposed to the same external in- 
juries at birth, and even if the disease is acquired later in life the two hemi- 
spheres seem often to be affected simultaneously, and much more frequently 
than in later years. 



434 



THE NERVOUS DISEASES OF CHILDREN. 



In my own statistics boys were affected a little more often than girls, but 
the difference is hardly great enough to be made much of, and Gowers has 
found just the reverse to be true. Osier found that girls and boys were 
affected in about equal proportion. 

The onset of a cerebral palsy will vary naturally according to the three 
groups which we have established. It would seem entirely superfluous to 
make any special remarks regarding the time at which the prenatal palsies 
begin. It might be sufficient to say that they begin during the intra-uterine 
period, and that such children are 
born palsied. It is a matter of fact 
that the symptoms of such intra- 
uterine cerebral defects are not al- 
ways manifest at birth, and indeed a 
number of months may pass before 
it becomes evident to the physician 
that the child's cerebral condition is 
not a normal one. A very fair per- 
centage of cases which appear to 
begin during the first years of life 
could properly enough be classified 
among the prenatal palsies, and if 
in any child the first symptoms of 
a cerebral palsy are noticed sev- 
eral months after birth, and yet the 
period of labor was entirely normal, 
I should be inclined to classify that 
case rather among the prenatal pal- 
sies than among the birth palsies. 

The birth palsies begin naturally 
enough with the period of labor. 
The history in these cases clearly 
shows that the labor was either ex- 
cessively prolonged, or that an in- 
strumental delivery was resorted to, 
in which case the brain has evident- 
ly suffered mechanical injury. Pre- 
mature delivery is responsible for 
many cerebral palsies ; but the symptoms may not be fully developed until 
months after birth. 

The age of onset in the acute or acquired forms varies considerably, and 
yet the chief statistics agree in this that fully two-thirds of all the cases be- 
gin in the first three years of life. Some maintain that by far the largest 
proportion begin during the first year, but I am not inclined to accept this 
view, since such a list would include a large number of cases which are more 
properly congenital, although the symptoms do not become manifest until 
some time after birth. Among a total of one hundred and forty cases, Pe- 
terson and I found that but five began in the eighth year, and four cases 




Fig. 106. — Right Hemiplegia, with Con- 
tractures and Retarded Growth of Arm. 
Onset of disease at eight years of age, 
following typho-malarial fever. 



INFANTILE CEREBRAL PALSIES. 435 

between the ages of fourteen and fifteen years, but these were cases of hemi- 
plegia, and it is of some interest to note that no patients with diplegia or 
paraplegia were observed after the third year, showing that the causes which 
lead to double cerebral lesions, and possibly the condition which permits 
double lesions, cease with the very earliest years of life. The majority of 
diplegias and paraplegias are either congenital or occur during the first year, 
which can be easily explained by the fact that the injuries sustained during 
labor are largely responsible for these special types. 



Etiology. — The true etiology of the prenatal cerebral 
palsies is often rather mysterious. We have to resort to 
vague statements that they are due to hereditary taints, and 
as a matter of fact such palsies occur frequently enough 
in families with a decided history of neurotic taints of 
one kind or another, with a history of hereditary epilepsy^ 
or of hereditary insanity. I have a number of times ob- 
served such children, in whom an hereditary taint was pres- 
ent in the families of both father and mother. The next 
most important factor is unquestionably the occurrence of 
some accident or injury to the mother during the period of 
pregnancy. This was to be traced distinctly in a number 
of cases of my own. The manner in which such trauma- 
tism may do actual injury to the brain of the child, was 
clearly shown by a case which was cited by Cotard. The 
mother of the child sustained a blow against the abdomen 
during pregnancy. The child, which was still-born three 
months after the injury, exhibited extreme contractures of 
both left upper and lower extremities. On examination of 
the brain an old lesion was discovered in the right hemi- 
sphere in the vicinity of the lateral ventricle. 

Illness of the mother during pregnancy, exhausting 
fevers, particularly such as accompany pneumonia and 
typhoid, uraemic convulsions, and severe fright, are other 
causes which have been made out in cases of prenatal 
palsies.* 

* Osier reports (in Teratologia, vol. ii., No. 1), upon the case of a young woman 
who died of typhoid fever. "The uterus contained a foetus, apparently about six 
months old. . . . The brain was very soft ; the right hemisphere was normal, but 
in the left . . . was a cavity with ragged, irregular walls, containing a large, recent 
clot, which had broken through the ganglia into the lateral ventricle of the same side. 
. . . No special changes were noted in the arteries." 



436 THE NERVOUS DISEASES OF CHILDREN. 

Hereditary syphilis does not play an important part in 
the etiology of prenatal palsies. Erlenmeyer, Osier, Oppen- 
heim, Koenig, and others mention it. As a matter of fact 
I have been able to convince myself of the influence of 
syphilis in but a single one of the many cases which I have 
seen. The fact that syphilis of the parents so frequently 
leads to still-births may account in a measure for the small 
part played by this special affection in these diseases. 

The etiology of birth palsies is a very simple one. It is 
surprising to note how much pressure the brain and skull 
will tolerate without injury, but it is natural that harm 
should occasionally be done. Asphyxia at birth has since 
Little's day been considered a most potent factor. My 
own studies in this matter, which were based upon a very 
careful collection of statistics, have proved that tedious 
labor is a more frequent and a more disastrous factor than 
instrumental delivery. Moreover, these birth palsies occur 
most frequently in first-born children. 

The prolonged compression of the skull during the last months of preg- 
nancy would seem to have exercised considerable influence in this direction, 
but, of course, the chief damage done is done during the period of actual 
labor. If physicians were more confident of the safety of the forceps and of 
their own skill to apply the same, protracted labor would not be as powerful 
an etiological factor as it is at the present time. There is, therefore, a distinct 
inference to be drawn from these facts, and a word of warning should be ut- 
tered to the obstetrician that, other things being equal, and, above all, the life 
of the mother not being in danger, it is wise to curtail the period of labor as 
much as possible, and not necessarily to wait until the child's heart action be- 
comes feeble. Many children might have escaped idiocy and epilepsy if the 
period of labor had been properly managed. 

Peterson is of the opinion that some, though a very small percentage (of 
birth palsies) may be due to cord lesions at birth. The same view has more 
recently been advocated by Dejerine. H. R. Spencer, among one hundred and 
thirty still-born children, found hemorrhage into the spinal canal and cord 
in thirty. Little suspected that " spinal meningitic and myelitic affections 
may play a considerable part in the phenomena of spastic rigidity " ; he also 
refers to a case of spastic rigidity, reported by Dr. Marion Sims, in which a 
coagulum of blood was found occupying the whole length of the spine. 

Acute cerebral palsies may be due to a variety of causes. 
The acute infectious diseases play a very important role ; 
measles, scarlatina, typhoid, small-pox, and even a tonsillitis 



INFANTILE CEREBRAL PALSIES. 437 

have been the precursors of such palsies. The same have 
also occurred after pneumonia and whooping-cough, but in 
the case of the latter it is questionable whether the palsy is 
not as often due to mechanical injury during a spasm of 
coughing as to the effect of the toxic agent. Fright — which 
Freud has interpreted to be equivalent to a psychic trauma 
— is an occasional cause of acute cerebral paralysis, but 
actual traumatic violence to the skull is a much more pow- 
erful factor. These palsies also occur after simple or cere- 
brospinal meningitis, after an exhausting gastro-enteritis, 
and after other severe fevers ; but, of course, there is always 
the danger of making a, post hoc a propter hoc. Among ninety- 
one cases of acquired cerebral palsy the exact cause could 
not be ascertained in twenty-seven. 

There has been a tendency, particularly among French 
authors, to claim that all cases of acquired cerebral palsy 
were due to acute infectious diseases. This seems to me 
to be straining the point altogether too much, nor is there 
much evidence that acute cerebral palsy in itself represents 
an acute infectious disease, as Striimpell claimed some years 
ago. As a matter of fact only relatively few of the cases 
of acquired cerebral palsy begin with fever, coma, and 
convulsions, the very symptoms which Striimpell thought 
most characteristic of polio-encephalitis. The writer has 
departed from his former views to the extent of conced- 
ing that a small number of the acute cerebral palsies of 
children may be due to an hemorrhagic (infectious?) en- 
cephalitis. 

The importance of convulsions as an etiological factor in acute cerebral 
palsies has given rise to some discussion. Freud and Rie are of the opinion 
that convulsions denote the onset of the cerebral process, but that they never 
hold a causal relation to the palsy which results from the cerebral lesion. I 
grant that in a very large number of cases this statement of the German 
authors is tenable, but in many others the palsy seems to be a more or less 
immediate result of the convulsive seizure. This view has been urged by 
Osier and myself. Anyone who has observed the marked disturbances of 
circulation at the acme of convulsions can readily conceive how easily a 
blood-vessel could burst during this period as a result of excessive stasis. 
This does occur, moreover, not only in children, but even in adults. I have 
the brain of a girl aged seventeen, who had had chronic epilepsy for years, but 
whose general health was not affected by the convulsive seizures ; after the 



438 



THE NERVOUS DISEASES OF CHILDREN. 



last attack which she had she became somnolent, comatose, and died after 
three days. Her brain was covered by a large subpial extravasation, which 
almost completely covered the entire left hemisphere and part of the right. 
If such an occurrence can take place in the brain of a girl of seventeen 
there is no sufficient reason why similar accidents of lesser extent should 
not happen frequently in the case of children. Ashby, not long ago de- 
scribed the brain of a child, twelve years of age, in which a number of old 
cysts were found which were probably the result of hemorrhages occurring 
during convulsions. There is clinical evi- 
dence also which cannot be neglected 
which tends to show that the convulsions 
constitute a decided danger to the child, 
and mark a turning-point in its entire 
career. I have still under my observation 
a child, now eighteen years, which I 
knew from the time of its birth, and which 
was entirely normal until the age of fifteen 
months. It had begun to stand, to walk 
a little, and had acquired some speech. 
It was in every way a healthy and men- 
tally vigorous child. At that age it was 
stricken down with chicken-pox, and had 
a single convulsive seizure, with a marked 
rise of temperature at the onset of this 
acute infectious trouble. From that time 
on the child's mental condition changed ; 
it lost its speech, and to this day, although 
it has grown physically, its mental condi- 
tion is that of complete idiocy. It would 
be a very remarkable instance indeed if 
an acute cerebral process had come on 
at exactly the same time as the chicken- 
pox. 

The Form of Palsy. — Hemiplegia, 
diplegia, and paraplegia are the usual 
forms of cerebral paralysis in children. 
Monoplegia, which we might expect on 
theoretical grounds, is, as a matter of fact, 
extremely rare. (Figs. 105-108.) 

The leg evidently recovers very much more quickly than the arm, as in 
adult hemiplegia, and for this reason it is a very great rarity to find a mono- 
plegia of the leg with few symptoms in the arm of the same side. Under 
the heading diplegia we may classify ail those cases in which both halves of 
the body have been involved, and it is better to attach the greatest impor- 
tance to this bilateral character of the palsy, even though the palsy be incom- 
plete, for the bilateral affection points to a double brain lesion, and that is 
the salient point in every such patient. In diplegia the legs may often be 




Fig. 107. — Congenital Diplegia — 
" Frog Girl." Double talipes 
equino varus ; athetosis of left 
hand ; right arm also weak ; in- 
telligence good. 



INFANTILE CEREBRAL PALSIES. 439 

much more affected than the arms, and some authors might be inclined to 
classify such palsies under the category of paraplegia. In a number of in- 
stances there has been a very complete spastic paraplegia associated with 
athetoid or choreic disturbances of motion in the upper extremities. This 
proves that the upper extremities were at one time involved, and for this rea- 
son I prefer to denote such conditions as an incomplete diplegia, or diplegia 
with partial recovery. Diplegias or paraplegias, in short double cerebral 
palsies, are, in the large majority of instances, due to a prenatal lesion, or to 
traumatism during labor, but I have been able to satisfy myself over and 
over again that both diplegias and paraplegias do occur in acute cerebral 
cases, and for this reason, in addition to others, I must protest against Striim- 
pell's views of making infantile hemiplegia synonymous with the acute cere- 
bral palsy of children.* 

The involvement of the face is a matter of some interest. In children that 
are observed long after the onset of the disease the face appears to be entirely 
normal, but in fully twenty per cent, of the acute forms which I have had an 
opportunity of examining, facial palsy was present in the earlier stages of the 
disease. I have never observed a double facial palsy in cases of diplegia. 
The reason of this is not far to seek, for the peculiar position of the facial 
centre is such that freshly exuded blood would not be apt to adhere to this 
part of the brain. Since the publication of my -own articles on this subject, 
and those of Freud and Rie, Koenig has called attention to the occurrence 
of mimetic facial palsies in children. The cases which he cites are very con- 
vincing, but this special form of facial palsy is surely very rare, or else it 
would scarcely have escaped the observation of so many authors. 

Aphasia is at times associated with acute cerebral palsy. It is invariably 
motor, not sensory in character. Of course aphasia will be developed only in 
those children who have acquired articulate speech before the onset of the 
cerebral palsy. This excludes from the list all diplegias and paraplegias 
which have come on before or during birth. There may be defective de- 
velopment of speech, but no aphasia in any true sense of the word. I have 
the records of at least seventeen children with hemiplegia and undoubted 
aphasia. Of these seventeen, ten occurred with right hemiplegia, and seven 
with left hemiplegia. Eight of these seventeen were observed by me in pri- 
vate practice, and of these eight, five have been distinctly aphasic, and three 
of the five were cases of left hemiplegia. It is of interest to note the relatively 
large proportion of aphasia in children with left hemiplegia. As we grow 
older the left hemisphere evidently obtains the upper hand, and after the 
earlier years of life we become, for all practical purposes, more and more 
left-brained. Bernhardt has come to the same conclusion that aphasia in 
children accompanies right as well as left hemiplegia. Aphonia has been 
recorded by some. 

Inasmuch as the cerebral palsies are due to lesions which involve other 

parts of the brain as well as the motor areas, it is not unreasonable to look 

for additional symptoms in these cases. Thus Freud was the first to call 

attention to the fact that hemianopsia was at times associated with the brain 

* The experience of more recent years has fully corroborated these opinions. 



440 



THE NERVOUS DISEASES OF CHILDREN. 



palsies of children. Oppenheim, Peritz, and some French observers have 
noted the occurrence of peculiar reflex actions — " Feed reflex " ; also an in- 
creased " motor acoustic reaction." The latter is identical with the " hypera- 
cusis " to which I directed attention many years ago. (See Amaurotic 
Family Idiocy.) 

The rigidities and contractures deserve especial no- 
tice. Some degree of con- 
tracture is present in fully 
seventy-five per cent, of the 
diplegias and paraplegias. 
The contractures occur 
early after the onset of the 
palsy, and evidently are de- 
veloped much more easily 
than in the cerebral palsies 
of the adult. It is interest- 
ing in this connection to re- 
fer to the case of Cotard, in 
which the palsy was due to 
an intra-uterine lesion, and 
the child was born with con- 
tractured extremities. The 
contractures may vary great- 
ly in degree and in the num- 
ber of joints affected. The 
flexors and pronators of the 
arm, the flexors of the legs 
and of the feet, are most fre- 
quently affected. In the cases 
of diplegia and paraplegia 
there is, in addition, a con- 
tracture of the adductors of 
the thighs, which is respon- 
sible for the peculiar cross- 
legged position of the legs 
and for the cross-legged progression if the child is at all able 
to walk. All these contractures give the child a characteris- 
tic position and gait by which we can recognize the trouble 
at first sight. Pes equinus or pes equino varus is the most 
common deformity of the foot. In a few cases an equino 




Fig. 108. — Case of Spastic Diplegia. 
Attempt to walk ; cross-legged pro- 
gression ; rigidity and paralysis of legs 
and of right upper extremity ; left up- 
per extremity weak. 



INFANTILE CEREBRAL PALSIES. 44 1 

valgus is present. If the upper extremity is contractured 
the arm is, as a rule, in close juxtaposition to the trunk, 
flexed at the elbow, and the hand is generally in a position 
of extreme flexion, the fingers often being firmly pressed 
into the palm of the hand. (See Fig. 106.) 

The gait varies much and is dependent both upon the paralysis and the 
degree of contracture. Many cases of hemiplegia in children have exactly 
the same walk as the adult hemiplegic patients have, but the peculiar cross- 
legged progression in cases of diplegia and paraplegia is characteristic of 
these infantile palsies, and has no counterpart in the cerebral palsies of the 
adult. In one case the contractures of the hip and knees were so extreme 
that the girl, who was otherwise well developed and bright, could walk only 
by skipping in the manner of a frog. She was baptized in my clinic as the 
"Frog girl." (Fig. 107.) 

The reflexes are almost invariably exaggerated, as can be expected from 
the fact that the lesion is in the first division of the motor tract. In a few 
instances only have I found the knee-jerk either normal or diminished. 
Babinski's reflex can be elicited in most cases, and Oppenheim's tibial reflex 
in some. (Dorsal flexion of foot and toes following upon stroking of inner 
side of leg.) In one child seen at my clinic the reflexes were increased in 
the upper extremities, and decreased in the lower, although the case was one 
of right hemiplegia ; on closer examination we discovered that two years after 
the onset of the cerebral palsy the child suffered an attack of polio-myelitis 
involving the right leg. This is, by the way, the only patient I have seen 
with a spinal lesion complicating cerebral disease. In some cases the reflexes 
appear to be absent, but this is generally due to a very marked contracture 
of the opposing muscles, which do not permit the excursion of the leg. Un- 
der such circumstances, however, even though the knee-jerk be wanting, the 
anterior thigh muscle can be seen to contract upon the tapping of the tendon. 
The ankle clonus and the triceps reflex are often inhibited for the reasons just 
stated. In other individuals, the reflexes are usually so much exaggerated 
that a single tap of the tendon is sufficient to produce clonic contractions of 
the muscle. 

The post-paralytic disturbances of motion constitute a very 
characteristic feature of cerebral palsies in children, and 
they follow with much greater regularity upon the infantile 
cerebral lesions than they do upon the brain lesions of the 
adult. That they are by no means uncommon in the brain 
lesions of the adult, I had occasion to observe several times, 
notably in an old woman, with a lesion in the crus, whose 
paralyzed arm performed the most violent ataxic move- 
ments. In children these post-paralytic disturbances of 
motion occur in fully one-third of all the cases. 



442 THE NERVOUS DISEASES OF CHILDREN. 

From a collection of statistics based upon one hundred and fifty-six cases 
of hemiplegia, and thirty-nine cases of diplegia, it was evident that athetoid, 
choreiform, and associated movements, are the most frequent disturbances 
to be noted. The choreiform and athetoid can generally be differentiated 
from one another, but in some cases there may be a combination of both. 
The athetoid movements are of especial interest, inasmuch as they are ob- 
served almost invariably in connection with the cerebral palsies, and it is 
questionable whether athetosis ever occurs as an independent disease, as was 
claimed by Hammond, who, however, deserves all the credit for the first vivid 
description of these peculiar movements. 

The associated movements are often developed to a startling degree, the 
paralyzed hand imitating all the movements of the normal hand. Thus a 
patient who is asked to button or unbutton his clothes, will imitate all the 
movements of the sound hand, by the paralyzed hand, although the latter 
may be held in mid-air. 

The choreiform movements occurring after cerebral pal- 
sies deserve some special notice, for such conditions are at 
times mistaken for cases of ordinary chorea. I have been 
consulted in not a few instances for what was supposed to be 
incurable St. Vitus's dance, which on examination has proved 
to be a form of infantile cerebral palsy, in which the palsy 
had largely disappeared, but the choreiform movements 
were left as evidences of the former more serious dis- 
turbance. In such children the presence of paralysis or of 
contractures, however slight, and the exaggeration of the 
reflexes in the choreic extremity, will be sufficient to indi- 
cate the nature of the choreic movements. 

Atrophy of the muscles plays an entirely negative part in the vast majority 
of infantile spastic palsies. Quincke, Borgherini, Darkschewitsch, Eisen- 
lohr, and others, have proved beyond a doubt that a cerebral muscular atro- 
phy does at times occur, but it is still questionable whether this is due to 
a lesion of the trophic centres in the brain, or whether the spinal gray mat- 
ter has not in some way been involved. I have observed a considerable atro- 
phy of the muscles in a few cases of diplegia and paraplegia. In one patient 
with paraplegia the atrophy was so extreme that the case would have ap- 
peared to have been one of spinal palsy if the presence of the reflexes and 
the electrical reactions, as far as any could be elicited, together with the en- 
tire history of the disease, had not pointed unmistakably to a cerebral lesion. 
While a true muscular atrophy does not occur, as a rule, the affected limb re- 
mains stunted in growth, and after a few years is considerably shorter than 
its fellow of the opposite side. Ibrahim and others have reported hyper- 
trophy of the paralyzed extremities, together with athetosis, but it has not 
been my good fortune to see more than one such case. 



INFANTILE CEREBRAL PALSIES. 



443 



Remak has reported a case of luxation of the shoulder-joint in an infantile 
cerebral hemiplegia, which he attributes to unequal innervation of the mus- 
cles surrounding the shoulder-joint. 

A distinct asymmetry of the body is present in many of the cerebral pal- 
sies, and to this we may add the asymmetric development of the skull, which 
has been shown to be present, by the careful measurements of Peterson and 
Fisher, in the majority of the subjects of infantile spastic hemiplegia ; but the 
skulls of such children are often not only asymmetrical, but also deformed in 
various ways. Some of them are microcephalic, others leptocephalic, doli- 
chocephalic, etc. Microcephalus is frequent enough to justify me in caution- 
ing the surgeon who may be ready to operate upon all such skulls that a 

small skull may harbor a dis- 
eased brain. Ibrahim has writ- 
ten on the association of micro- 
cephalus with infantile spastic 
diplegia, and believes that this 
association points to the pre- 
natal origin of these diplegias. 

The most serious of 
all the symptoms asso- 
ciated with infantile 
cerebral palsy is epi- 
lepsy. A number of 
children have been 
brought to me with the 
diagnosis of epilepsy, 
pure and simple, in 
which this condition 
was the outcome of 
the same cerebral dis- 
turbance which gave rise to the palsy. The palsy may dis- 
appear, or may be so slight as to give little inconvenience 
to the child, whereas the epilepsy unfortunately remains. 
This association is so common that I am inclined to the view 
that a fair percentage of the cases of supposed genuine 
epilepsies may be attributed to cerebral lesions which oc- 
curred in early childhood, and have given rise to cerebral 
palsies. I am glad to see that this view has been quoted 
approvingly by a number of authors, among others by 
Freud, who has unquestionably given this entire subject 
the most careful study. A single case will bring out the 
truth of this very clearly. 




Fig. 109. — A Child with Congenital Diplegia and 
a Microcephalic Skull ; Strabismus and Idiocy. 



444 THE NERVOUS DISEASES OF CHILDREN. 

Some years ago I was asked to see a girl, seventeen years of age. A 
number of able physicians had treated her for genuine epilepsy. Upon ex- 
amination of the girl I was astonished to find that the left extremities were 
weaker than the right, and that the deep reflexes of the left upper and lower 
extremities were considerably exaggerated. Upon questioning the mother 
closely I discovered that four years previously the girl had had a slight apo- 
plectic seizure, after which the convulsions first appeared. The palsy had 
diminished to such an extent that everyone would have considered the gait 
of the girl entirely normal. Her mental condition was impaired by the 
enormous doses of bromide which had been administered in the hope of 
curing the epilepsy. Recognizing its organic origin, I decided to discon- 
tinue the bromides, and, as a result, the epilepsy is no worse and no better ; 
but the girl's mind has considerably improved, and the anaemia, which was 
aggravated by the administration of the bromides, has been considerably 
diminished. 

The first epileptic attacks associated with a cerebral palsy may occur very 
soon after the onset of the disease, and may be repeated a number of times 
during the first week or two. After this a lull sets in, but unfortunately the 
attacks often recur after several months, sometimes after a year or more, 
when it is positive that a chronic epilepsy has been developed. According to 
my own statistics, fully forty-five per cent, of all the infantile cerebral palsies 
develop epilepsy. This occurs, furthermore, in about fifty per cent, of the 
cases of hemiplegia, in about thirty per cent, of all forms of diplegia, and in 
about thirty-six per cent, of patients with paraplegia. I have furthermore 
observed that the epilepsy does not necessarily develop according to the 
severity of the palsy, for some of the worst cases of epilepsy I have seen have 
been observed in cases in which the palsy was extremely slight. The corti- 
cal origin of infantile cerebral palsies is sufficient reason for the more fre- 
quent development of epilepsy after infantile than after adult cerebral palsies ; 
and if the character of the palsy is such that the lesion in the child can be 
proved to be capsular rather than cortical, the prognosis as regards epilepsy 
may be considered favorable. Jacksonian epilepsy is present in some of the 
palsies, and, if present, can easily be explained on physiological grounds, but, 
as a matter of fact, it is not observed as commonly as one would suppose, 
for the simple reason that the majority of patients have been examined after 
several years have elapsed, and the unilateral order of convulsions has been 
superseded by a general convulsion. Koenig has given close study to this 
part of the subject. 

Idiocy is developed very often with epilepsy and with 
some forms of cerebral palsy. Every possible variation of 
mental deficiency may be present, from slight mental en- 
feeblement to a complete idiocy. The severer forms are 
oftener present in the cases of diplegia and paraplegia than 
in hemiplegia, and naturally enough, for in the former there 
is a double lesion or disease of both halves of the brain 



INFANTILE CEREBRAL PALSIES. 445 

which will disturb the mental development far more than a 
unilateral lesion would. 

I have found idiocy present in thirty-five per cent, of all diplegias, and in 
sixty per cent, of all paraplegias, while it occurred in but thirteen per cent. 
of the subjects of hemiplegia. It was this very high percentage of idiocy in 
cases of diplegia and paraplegia that forced the conclusion upon me, some 
years ago, that these congenital paraplegias must be of cerebral rather than of 
spinal origin. There is no telling in advance whether, in a given case, idiocy 
will develop or not, for, unfortunately, relatively slight paralysis is sometimes 
complicated by severe idiocy. Marie claims that there is no disturbance of 
intelligence in cases of double athetosis. I must insist that there are excep- 
tions even to this rule. The frontal lobes are supposed to be silent parts of 
the brain, but the defect in intelligence tells a sadder tale of its important 
functions than the palsy does of the functions of the motor centres. 

Various attempts have .been made to classify the cere- 
bral palsies of children, and to separate them into distinct 
clinical groups. The classification according- to the period 
of development, which we have adopted, is, on the whole, 
the most practical one. The palsies due to intra-uterine 
disease or to arrest of development, and those due to 
traumatism during labor, give a distinct history of early 
onset of all the symptoms. In these children bilateral pal- 
sies (diplegia and paraplegia) are more common than in the 
acute cases, and there is often a very decided defect in 
mental development. The paralysis is not necessarily com- 
plete ; at times it is a simple paresis, at other times rigidi- 
ties and contractures are more prominent than the palsies 
are. English and American authors have not drawn any 
sharp clinical lines, as all these forms, although they may 
vary a little, seem to merge into one another, and are evi- 
dently due to similar morbid processes. Little distinguished 
between rigidities and palsies, and several German authors 
have adhered to this distinction ; but one of the ablest of 
them, Freud, * has practically adopted the American point, 
of view. Rigidit}^, contracture, choreic or athetoid move- 
ments, paresis, paralysis (unilateral or bilateral), constitute 
a graduated series of disturbances due to cerebral lesions 
in children. Each one, or a combination of all of them, may 

* This author subdivides the diplegias into four groups : 1. Universal spastic rigidity; 
2. paraplegic rigidity ; 3. bilateral hemiplegia ; 4. bilateral chorea and bilateral athetosis. 



44^ THE NERVOUS DISEASES OF CHILDREN. 

be present in a given case, and may vary according to the 
site and intensity of the lesion. The best proof that all these 
disturbances of motility are closely related is, that in certain 
cases of diplegia there is paralysis in the lower extremities 
and athetosis in one or both upper extremities. Double 
athetosis is evidently a condition closely analogous to the 
double hemiplegia, with less paralysis than in other cases. 
Freud showed that there was a form of acute cerebral palsy 
with little palsy but distinct chorea ; he described this con- 
dition as one of choreiform paresis, and recognized an early 
and a late form. Unilateral and bilateral chorea and athe- 
tosis may be developed in the place of a palsy, or the palsy 
may come first and the chorea and athetosis may represent 
post hemiplegic disturbances of motion. 

The diplegias and paraplegias are generally due to ar- 
rest of development or to abnormal birth conditions. (Some 
distinctly hereditary and family affections allied to these 
forms will be discussed at the close of this chapter.) Charcot, 
.Marie, Dejerine, van Gehuchten, and Spiller insist that con- 
genital spastic paraplegia may be a spinal affection and due 
to defective development of the pyramidal tract. For a time 
Ross adhered to a similar view ; but the defect surely in- 
volves the brain in a large majority of instances, or else we 
could not account for the high percentage of idiocy among 
children with congenital paraplegia. It is in this group of 
diseases that thoroughly satisfactory post-mortem examina- 
tions are needed. One case of Foerster and one of my own 
have been relied upon. Recently Dejerine has made an 
interesting contribution to this subject. 

Morbid Anatomy. — The study of the morbid lesions 
underlying these cerebral palsies in children will be facili- 
tated very much by considering separately the three chief 
groups of cases. A reference to the following table will 
show that in the prenatal cases large cerebral defects are 
often found. Porencephaly is present in its widest sense. 
Half or even more of an entire hemisphere, or of both 
hemispheres, may be wanting, or a considerable portion of 
one hemisphere may be poorly developed ; in such cases, too, 
as a rule, the palsy which exists is but one of a long series of 
symptoms, among which idiocy is by far the most promi- 



INFANTILE CEREBRAL PALSIES. 



447 



nent. If it were not for the fact that porencephalic defects 
occur more frequently in the motor areas of the brain than 
in any other part, we would scarcely be entitled to associate 
porencephaly more especially with these cerebral palsies. 

Classification of Infantile Cerebral Palsifs. 



Groups. 


Morbid Lesion. 


I. Paralyses of intra-uterine 


Large cerebral defects. (Porencephaly). 
Defective development of pyramidal tracts (p. 446). 




Agenesis corticalis. (Highest nerve elements in- 
volved ) 


II. Birth Palsies \ 


Meningeal hemorrhage, rarely intra-cerebral 
hemorrhage. Later conditions : Meningoen- 




cephalitis chronica, sclerosis, and cysts ; partial 
atrophies. 


IIL Acute (acquired) Palsies 


Hemorrhage (meningeal, and rarely intra-cere- 
bral) ; thrombosis (from syphilitic endarteritis 
and in marantic conditions ; embolism. Later 
conditions : Atrophy, cysts, and sclerosis (dif- 
fuse and lobar). 

Meningitis chronica. 

Hydrocephalus (seldom the sole cause). 

Primary encephalitis ; polio - encephalitis acuta 
(Striimpell). 



In addition to these large defects in cerebral develop- 
ment, other changes occur in the brain which are not quite 
as conspicuous, but are fully as effective in the way of pro- 
ducing serious symptoms and serious conditions during life. 
This condition has been properly designated " agenesis cor- 
ticalis." It implies a defective development of the cellular 
elements of the cortical, and particularly of the pyramidal, 
cells, and is not restricted to any one part of the cortex, but 
involves all parts of the hemispheres about equally. This 
condition is present in central amaurotic idiocy, associated 
with similar changes in the gray matter. 

The morbid lesions in birth palsies have been satisfacto- 
rily cleared up within the past few years. We owe much 
of this knowledge to the successful demonstration by Dr. 
Sarah McNutt of a case in which there was wide-spread men- 
ingeal hemorrhage in consequence of traumatism during 
labor. (Fig. no.) Dr. McNutt furnished positive proof 
of a condition which Little suspected years ago, in attribut- 



44* 



THE NERVOUS DISEASES OF CHILDREN. 



ing these palsies to the difficulties during birth. There 
can be no doubt but that in a vast majority of cerebral birth 
palsies meningeal hemorrhage, more or less diffuse over 
one or both hemispheres, is the direct cause of the disease. 

The failure to recognize this simple condition has been due to the fact 
that the cases examined for the purpose of determining the morbid states 
underlying these palsies have lived for a number of months, or even a year, 
and at the end of that time the character of the initial lesion cannot be 
recognized. A very instructive case of this description is the one referred to 




Fig. iio. — Meningeal Hemorrhage at Birth. Death on the twenty-second day. 

(McNutt.) 

in a previous chapter (page ). The child was born asphyxiated after 
forty-eight hours continuous labor pains, and an absolutely dry labor at that. 
From the very first day of its life until the age of six months, when I first 
saw the child, it had innumerable epileptic spells. The child died at the age 
of one year. The autopsy and a careful microscopic examination revealed a 
chronic meningo-encephalitis. This condition was in all probability due to a 
meningeal hemorrhage, though the traces of the hemorrhage had so thor- 
oughly disappeared that it was difficult to prove this with absolute certainty. 
In some instances an original meningeal hemorrhage, particularly if it be 
more or less circumscribed, may lead to the formation of a limited atrophy, 
in which case a condition resembling porencephalus may be the result. A 
few years ago Kundrat described a condition of minute hemorrhages from the 
meningeal veins, which he considered to be caused by a compression of the 
longitudinal sinus, in consequence of the displacement of the parietal bones. 



INFANTILE CEREBRAL PALSIES. 449 

Such hemorrhages Kundrat thought were found rather frequently in the brains 
of healthy children. It is doubtful whether we can connect this form of 
venous hemorrhage with the subject which we are now considering. 

The amount of damage done during labor can be gauged 
to a certain extent by the symptoms during the first few 
days of life. If there is wide-spread meningeal hemorrhage 
convulsions set in at a very early period ; the child is apt to 
be paralyzed at once, and is also in danger of lapsing into 
coma. Fortunately a number of the severer cases die 
promptly within a few days, but others again survive, and 
not a few of these become hopelessly crippled for life. Ac- 
cording to the amount of injury done, and according to the 
special parts of the brain covered by the hemorrhage, the 
form of the palsy, the degree of mental enfeeblement, and 
the severity of the epilepsy will vary not a little. 

The morbid anatomy of the acute cerebral palsies has 
given rise to the largest amount of discussion. Hemor- 
rhage, embolism, and thrombosis, the conditions which give 
rise, in the vast majority of cases, to the apoplexy of the 
adult, are important factors also in the causation of the acute 
cerebral palsies of children. This conclusion was forced 
upon us and others by a careful review of the autopsies 
made by different authors. 

Peterson and myself analyzed the records of seventy- 
eight autopsies in infantile hemiplegia, as follows : 

Lesions. Number of Cases. 

Atrophy, sclerosis, and cysts (terminal conditions) 40 

Porencephalus 2 

Hemorrhage 23 

Embolism 7 

Thrombosis 5 

Tubercle 1 

Total 78 

In 35 cases of hemiplegia hemorrhage occurred in 23, 
embolism in 7, and thrombosis in 5 cases. Professor Osier, 
who examined 90 brains, found a vascular lesion in 16 only, 
and among these 16, 7 were due to hemorrhage, and 9 
to embolism ; but we must not forget that a large number 
of the cystic conditions may be due to hemorrhage or em- 



450 THE NERVOUS DISEASES OF CHILDREN. 

bolism, and that many a case which is quoted as typical 
atrophy or porencephalus may also belong to the same 
category. There has been some little difference of opinion 
among recent writers on this subject with regard to the 
relative frequency of these various conditions. Some are in 
favor of the greater frequency of embolism ; others, like my- 
self, believe that hemorrhages are most commonly the cause 
of these conditions ; while according to Gowers thrombo- 
sis of the superficial veins is a very noteworthy initial lesion. 
The probability is that all these accidents occur, and that 




Fig. ill. — Cyst formed by Softening of Brain Substance, secondary to Obstruction of 
the Middle Cerebral Artery beyond the First Branch (to Inferior Frontal Convolu- 
tion). The cyst wall has fallen in from escape of its contents. Child nineteen 
months old. Death seven months after onset of paralysis. (After Ashby and 
Wright.) 

there is no satisfactory reason for claiming more for the 
one than for the other finding ; but with regard to the occur- 
rence of hemorrhages it is fair to inquire what morbid con- 
dition of the blood-vessel leads to the frequent occurrence 
of hemorrhage in children. 

In my endeavors to elucidate this part of the inquiry I have been compelled 
to fall back upon the fatty degeneration of the blood-vessels which Reck- 
linghausen describes in his masterly Monograph, and which he claims is a 
not uncommon finding in the brains of children. For the present we can do 
no better than to adhere to this view of Recklinghausen, and to accept it as a 
partial explanation, at least, of the frequent occurrence of hemorrhage. Some 
cases are of course explained by the more delicate structure of the blood-ves- 



INFANTILE CEREBRAL PALSIES. 



451 



sels in children as compared with the adult. But there is still another striking 
difference between these infantile and adult apoplexies. In the adult the 
majority of hemorrhages occur in the vicinity of the internal capsule. In the 
child they occur in or near the cortex. Mendel has shown that the greatest 
pressure is exerted in the branches of the middle cerebral artery, and that 
any increase of pressure would naturally cause a rupture in one of these. 
We would have to seek some other explanation for the frequent occurrence 
of cortical and meningeal hemorrhages, and perhaps they will be sufficiently 
accounted for by the more delicate structure of these vessels in children. Oc- 
casionally intra-cerebral hemorrhages, embolism, and thrombosis occur in 







;,y 






'.?* 



Fig. 112.— Section through Portion of Motor Cortex, removed by Dr. Gerster during 
an Operation for Localized Epilepsy, associated with Old Left Hemiplegia in a Boy 
Twelve Years of Age. Specimen was stained according to Van Gieson's method. 
The pia, P, which is greatly thickened and altered, dips down between two folds of 
the cortex, C. B, increased number of thickened small arteries ; just above, to the 
right, a large artery with thickened walls. H points to a recent clot. The line, a b, 
denotes the part of cortex examined under higher power and represented in Fig. 146. 



children in exactly the same manner in which they occur in the adult, and 
they give rise to clinical symptoms in nowise different from those of ordinary 
apoplexy. An interesting case of this sort I had occasion to observe in a boy, 
two and a half years of age, who after a simple attack of tonsillitis developed 
right hemiplegia with motor aphasia, without coma and without convulsions. 
The aphasia disappeared after a few days, and the hemiplegia also improved; 



452 



THE NERVOUS DISEASES OF CHILDREN. 



within a period of a few weeks the young boy was entirely well, and scarcely 
retained a trace of the apoplectic condition. There was no history of syph- 
ilis in the case, and the entire development and retrogression of the symp- 
toms reminded one of an adult apoplexy. The possibility of thrombosis 
could not be excluded, but whether hemorrhage or thrombosis, there can be 
no doubt about this, that the lesion was intra-cerebral and not cortical, as in 
the majority of cases in children. Since my own case was published, De- 
jerine. in 1891, reported three cases in 
wnich there were distinct hemorrhagic 
lesions in the vicinity of the larger gan- 
glia. 



Heart disease, rheumatism, 
scarlet fever, and pneumonia, 
are the conditions which pre- 
dispose to embolism. Throm- 
bosis may be suspected in cases 
of children dying of marasmus, 
but under such conditions the 
existence of a palsy, is, as a 
rule, overlooked, and of little 
practical importance. 

Thrombosis may also be the result of 
arterial changes due to hereditary syph- 
ilis, but I wish distinctly to impress upon 
the reader that in these cases of cerebral 
palsies syphilis does surely not play as 
important a role as is imputed to it by a 
few authors, who have generalized from 
the examination of one or two cases.* 
Gowers has advanced important reasons 
for the supposition that thrombosis and 
occlusion of the middle cerebral veins are 
of frequent occurrence in children, and 
that some of the cases of infantile hemi- 
plegia would be more likely to be due 
to this condition than to embolism re- 
sulting from endocarditis. He states, 
furthermore, that the thrombosis cannot 
be distinctly demonstrated post-mortem, 
because the thrombosis is, as a rule, 

continued into the sinuses, and a sinus thrombosis would be much more. 

striking, and would be apt to conceal the venous thrombosis which led to it. 

* I have had no reason to change these views. 




Fig. 113. — Variously Degenerated 
Cells of the Cortex in the vicinity 
of the line a b of the preceding fig- 
ure. Near a, small blood-vessels, 
walls thickened. Large pyramidal 
cells are misshapen, and exhibit 
granular disintegration. Cells di- 
minished in number. (Drawn from 
specimen.) 



INFANTILE CEREBRAL PALSIES. 



453 



Whatever the initial lesion of an acute cerebral palsy 
may be, if the patient survive a number of years, secondary 
changes may be set up in the brain which will successfully 
conceal the initial lesion. Cysts, large areas of softening, 
atrophy, sclerosis (diffuse and lobar), are a few of the 
changes frequently observed. 

There is no telling in advance of the post-mortem examination, with any 
degree of certainty, which secondary lesion will be found in the brain of a 




Fig. 114. — An Old Hemorrhagic Cyst. The cyst walls have been cut to expose tu- 
mor underneath (compare with Fig. 150). Right hemiplegia at age of six and a half 
years ; death two years later. 

child that has been afflicted with an acute cerebral palsy for a number of 
Tears. Cysts are so frequent that I have in a number of instances been able 
to predict the presence of this condition, particularly in those cases in which 
there was little idiocy associated with the palsy, and in which everything 
seemed to point to an initial lesion of considerable intensity, but limited in 
extent. And in several other cases that came under my own notice for opera- 
tion the surgeon found a cyst in the motor area, which I had predicted to 
be the probable condition. 

Various forms of sclerosis are among the most frequent 
sequelae of the initial lesions of infantile cerebral palsies. 



454 THE NERVOUS DISEASES OF CHILDREN. 

This sclerosis evidently starts from a focal region and rap- 
idly speads throughout the brain. If we bear in mind that 
the hemispheres are traversed by innumerable fibres which 
are intimately connected with one another, we can under- 
stand why a sclerosis should follow upon a relatively small 
focus of disease, just as secondary degeneration follows 
upon a lesion in the motor area, and affects all the fibres 
transmitting centrifugal impulses. This sclerosis, which 
develops after an initial lesion, is largely responsible for the 
symptoms which are so frequently associated with these 
palsies. I refer to idiocy and epilepsy. 

In the table given at the beginning of this discussion I have, for the sake 
of completeness, inserted chronic meningitis as an occasional cause of an ac- 
quired cerebral palsy. It is not entirely accurate to attribute a cerebral 
palsy to a chronic meningitis, but it is better to say that both the chronic 
meningitis and the cerebral palsy are the result of a cerebro-spinal or of a 
convexity meningitis which occurred early in life, and which the child sur- 
vived. These cases are sometimes to be differentiated from others by the 
persistent paralysis of various cranial nerves in addition to the paralysis of 
the extremities. 

The reader may be surprised to find polio-encephalitis acuta given as the 
very last morbid condition underlying acute cerebral palsy. Striimpell's idea 
was a fascinating one indeed, and it is to be regretted that later post-mortem 
findings did not support his theory. The analogy which this author drew 
between the infantile spinal paralysis and the infantile cerebral palsies was 
based upon clinical resemblance, but we well know that symptoms which are 
scarcely to be differentiated from one another may be due to a variety of 
morbid processes. Striimpell maintained that there was a close clinical and 
pathological relation between polio-myelitis and polio-encephalitis acuta. 
More recently he has modified his views, and now claims that acute encepha- 
litis of the gray, as well as of the white matter, may constitute the basis for 
these acute cerebral palsies in children. The actual proof which Striimpell 
offered for his views was based upon the occurrence of two cases in one 
family, reported by Moebius, in which two children were affected at one and 
the same time ; the one with a typical polio-myelitis, and the other with a 
spastic cerebral palsy without atrophy. This may have been a mere coinci- 
dence, and further evidence must be forthcoming before we can accept this 
as proof of the theory. Striimpell himself refers to two cases in the adult in 
which the diagnosis of hemorrhage was made during life, and in which, at 
the post-mortem examination, acute encephalitis was found. From this we 
may infer the possibility of a similar occurrence in children, but until such a 
condition can be satisfactorily established there is no good reason to accept 
encephalitis as more than the rarest of all causes that give rise to infantile 
cerebral palsies. A case of (supposed infectious) encephalitis of the island of 



INFANTILE CEREBRAL PALSIES. 455 

Reil, reported by Felsh, is not convincing. Lesions affecting the motor areas 
give rise to a palsy ; similar lesions may affect silent parts of the brain, and 
the result of such lesions may be a general cerebral disturbance, defective in- 
telligence, or chronic epilepsy. The fact has been well emphasized by Freud 
who speaks of cases of " cerebral palsy without palsy." 

Differential Diagnosis. — Infantile cerebral palsies 
are frequently confounded with the acute spinal affections. 
The cerebral cases are characterized by the hemiplegic, 
diplegic, or paraplegic form of paralysis, by spastic rigid- 
ities and contractures, by increase of the deep reflexes, by 
the entire absence of any considerable degree of atrophy, 
and by entirely normal electrical reactions. Anyone who 
is able to recognize the difference between lesions in the 
first and second divisions of the motor tract will be able 
to distinguish between the cerebral and spinal forms of in- 
fantile palsies. Difficulties may, however, arise in the cases 
of mild types of cerebral palsy in which the spasticity and 
contractures may be but slightly developed, and the re- 
flexes may not be very much increased ; but even under 
such circumstances the hemiplegic distribution of the palsy 
and the entire absence of all changes in the electrical reac- 
tions will help to demonstrate the cerebral character of the 
disease. In other patients, the presence of athetoid or 
choreic movements in one half of the body, with slight 
exaggeration of the reflexes, will indicate a former cere- 
bral palsy, the paralysis having wellnigh disappeared. Spi- 
nal and cerebral palsies may occur at different periods of 
a child's life, but I have seen only one instance of this 
among the large number of cases that have come under my 
notice. The spastic paraplegia due to traumatism during 
labor is to be differentiated from hereditary spastic paraly- 
sis (see Chapter XXI.) and from syphilis of the spinal cord 
(see Chapter XVII). 

Although the diagnosis is simple enough, the knowl- 
edge of these infantile cerebral palsies is still so very imper- 
fect among physicians that cases coming under this cat- 
egory are frequently overlooked. I would particularly 
urge the examination of patients with peculiar forms of 
athetosis or chorea in order to determine whether these 
are not associated with some traces of a preceding cerebral 



456 THE NERVOUS DISEASES OF CHILDREN. 

palsy. If such children exhibit hemiparesis with slight 
defects in mental development, or with occasional epileptic 
attacks, the true nature of the disease can readily be dis- 
cerned. 

Prognosis. — In the congenital forms of cerebral palsy 
the prospects as regards life and the normal development 
of the child cannot be formulated until after a period of 
some weeks or months. It is a fortunate circumstance on 
the whole that children who come into the world with a 
severe cerebral lesion succumb to it at a very early period, 
but of those who survive a fair proportion develop idiocy 
as well as epilepsy, and are frequently permanently crip- 
pled. If convulsions occur during the earlier weeks of 
life the severity of the lesion may be inferred from this 
fact. If such a child, moreover, show no mental awakening, 
the probability of more or less complete idiocy is very 
great indeed. If, after a few weeks or months, the convul- 
sions are noticeably diminished, if the child shows any ten- 
dency to a tolerably normal use of its legs, and if it begins 
to take notice of its surroundings, a more favorable prog- 
nosis may be given. As long as contractures do not de- 
velop the child may acquire a fair use of its extremities. 
Diplegia and paraplegia are more apt to be associated with 
cerebral deficiency and epilepsy than is unilateral palsy. 

In the acute cases there is much uncertainty for some 
time after the onset of the attack, and no definite statement 
is warranted regarding the permanency of the paralysis, or 
the mental condition of the child until several weeks have 
elapsed, when it will be seen whether there is any tendency 
or not to recovery. If a child with an acute form of infan- 
tile hemiplegia shows some improvement after a few weeks, 
either in the leg or in the arm, and if the speech that was 
lost begins to return, there is every reason to be hopeful 
regarding the ultimate outcome of the disease ; but if weeks 
and months pass without any such favorable change the 
probability of permanent crippling of the mind and body is 
very great. It is, however, a matter of common experience 
that the child may do extremely well for a number of 
months, for a year, or even longer, after an attack, when 
convulsions may reappear and the epileptic habit may be- 



INFANTILE CEREBRAL PALSIES. 457 

come established. I have paid considerable attention to 
this point, and find that among the cases examined by me 
epilepsy occurred in fully forty-five per cent, of all the 
cerebral palsies, and nearly one-half of the cases of cerebral 
hemiplegia developed epilepsy at a later period. It would 
seem from this that the unilateral brain lesion leads to epi- 
lepsy more frequently than the double lesion does, for ac- 
cording to the same statistics epilepsy is present in about 
twenty-nine per cent, of the diplegias, and in about thirty- 
six per cent, of the cases of paraplegia. My own results 
regarding the development of epilepsy are corroborated by 
the statistics of Gaudard, Osier, and Wallenberg. The 
fact that fewer cases of diplegia and paraplegia survive 
may account for this apparent difference in favor of the 
double lesions. If a child that has passed through an 
apoplectic seizure, and has some form of congenital paraly- 
sis is taken with convulsions, it is more than probable that 
such convulsions are the first of a series that may lead to 
the development of chronic epilepsy, and from this time on 
the prognosis becomes extremely grave. 

Treatment. — The treatment of infantile cerebral pal- 
sies is not altogether devoid of interest. These diseases 
call for deliberate non-interference at the start, and for 
sober judgment in therapeutic matters during the later 
stages. The treatment of the later stages "will be prac- 
tically the same whether a case be congenital or acquired. 
If a child but a few days old exhibits a tendency to drow- 
siness or to convulsions its brain has in all probability sus- 
tained a serious injury during labor. It should be kept 
quietly in its crib or bed, and the greatest attention should 
be paid to its nutrition. These children are often unable 
to swallow and unable to suckle at the breast, and for this 
reason must be carefully fed with a spoon. Milk, properly 
prepared, according to the age of the child, is the very best 
nourishment. If there is a tendency to convulsions, or if 
marked convulsive seizures occur, the child may be given 
very minute doses of bromides, or minimum doses of mor- 
phia or chloral. A drop of the solution of Majendie, or a 
grain of chloral per rectum, will be quite sufficient in the 
case of a new-born babe. If convulsions continue in spite 



458 THE NERVOUS DISEASES OE CHILDREN. 

of these measures, inhalations of chloroform may be prac- 
tised, however young the child may be, but of course with 
the greatest possible care. All other measures, such as 
counter-irritation, mustard-baths, and the like, are practi- 
cally useless. 

In the initial stages of an acute cerebral palsy the same 
measures should be employed which the physician would 
resort to in the case of an adult apoplexy, allowing always for 
the difference in the ages of the patients. Absolute rest is 
the first condition of treatment. An ice-bag may be ap- 
plied to the nape of the neck, or to the head, and if it do no 
good, it will at least do little harm. The use of ergot or of 
the nitrite of amyl, as has been advocated in adult apoplexies, 
I cannot favor. The fewer drugs that are administered the 
better it will be, and the physician will do well to limit 
himself entirely to the administration of calomel in doses suf- 
ficient to procure free purging of the bowels, and small doses 
of bromide, which may be given in order to secure rest for 
the disturbed brain. At a later period the bromides may 
be combined to advantage with the iodides, but in every 
case in which the digestion or the general nutrition suffers 
from the administration of these drugs, their use should be 
prohibited for the time being ; for the good that they can 
effect will not counterbalance the evils that follow upon a 
gastritis and its attendant malnutrition. 

As soon as the symptoms of the initial period have 
passed, the physician is compelled to prescribe some form 
of treatment for the paralysis and the other symptoms of 
the disease. The paralysis is the natural result of the lesion, 
and cannot of course be removed by any therapeutic meas- 
ures. It is well to explain this distinctly to the parents 
and relatives, and to state at once the chronic nature of 
these troubles, and the length of time that will probably 
elapse before any great change can be expected to take 
place. As soon as the active period of the disease has 
passed, massage and electricity should be applied to the 
paralyzed parts, but let the brain severely alone. The 
effect of electricity upon the circulation within the skull is 
altogether too uncertain to justify us in tampering with it in 
any way. Electricity applied to the extremities can do no 



INFANTILE CEREBRAL PALSIES. 459 

harm, and serves an excellent purpose as a means of exer- 
cising parts that cannot be moved by the will. As these 
parts respond to the faradic as well as to the galvanic cur- 
rent, I am in the habit of using the former chiefly, and of 
using a current strong enough to produce mild contrac- 
tions. Powerful currents should be avoided. The electric- 
ity should be administered in sittings from ten to fifteen 
minutes every day, or every other day, and should be given 
by the physician, or by an extremely competent nurse 
under the guidance of the physician. If passive movements 
are combined with ordinary friction the paralyzed parts will 
be kept in a good state of nutrition, and the tendency to 
contracture may be overcome. 

As in the cases of spinal paralysis, so in these cerebral palsies, orthopedic- 
measures should be resorted to as soon as contractures have become perma- 
nently established. I have seen many a child walk about fairly well a few 
weeks after the orthopedic surgeon has been allowed to cut the tendons, and 
to provide the proper orthopedic appliances. Contractures, if once formed, 
are rarely, if ever, recovered from in spontaneous fashion, and if it can be 
proved that the contractures are the chief hindrance to the child's progress in 
walking, full liberty should be given to the orthopedic surgeon ; Eulenburg, 
Hoffa, and others have obtained good results by tendon-transplantation. I 
have been especially pleased with what Dr. Gibney has been able to do in 
several cases of marked disturbances of the upper extremities that have oc- 
curred in cases of infantile cerebral palsy. I refer in particular to the persist- 
ent athetoid-choreic movements of the upper extremities so common in these 
cases. By the application of some simple form of restraint such post-para- 
lytic movements may be successfully inhibited. In the case of a young man, 
seventeen years of age, who had acquired a cerebral palsy when he was ten 
months old, and who had exhibited very annoying athetoid movements of 
the upper extremities from that time on, the simple splint arrangement which 
prevented the possibility of these movements, and which was worn for a 
number of months, was sufficient to inhibit the athetoid movements altogether ; 
and in still another case a marked post-hemiplegic chorea was much im- 
proved, if not altogether inhibited, by placing the arm in a plaster-of-Paris 
bandage for a period of two months. 

The treatment of the idiocy must be conducted on the same lines as in 
other forms of idiocy. 

By far the most important task is the treatment of the 
epilepsy associated with the palsy. We have stated else- 
where that this epilepsy is due to the secondary changes in 
the brain, which have developed as a natural result of the 



460 THE NERVOUS DISEASES OF CHILDREN. . 

original lesion which gave rise to the palsy. In all these 
cases the epilepsy is practically a focal epilepsy, although 
its clinical manifestations are not often of the strictly Jack- 
sonian order. It was quite natural that Horsley's sugges- 
tions with regard to the proper treatment of focal epilepsy 
should be applied to these forms of epilepsy with infantile 
palsies. 



Horsley himself has reported upon two such patients, and in both of these 
a cystic condition was found. Freud reports a case of Oppenheim's in which 
a meningeal cyst over an arm centre was evacuated, but the brain itself was 
not operated on by the surgeon ; the athetosis and epilepsy in these subjects 
were considerably diminished. The author has had several cases of cerebral 
palsy with epilepsy operated upon. The first case, a boy, six years of age, had 
acquired right hemiplegia at the age of five, and soon thereafter developed 
convulsions, which invariably began in the right hand. This right hand also 
exhibited athetoid movements. Dr. Gerster trephined over the motor centre 
for the right arm, and exposed the greater part of the arm and leg centre in 
the left half of the brain. The boy did well for three months after the op- 
eration ; his attacks disappeared altogether, but the ultimate result cannot be 
stated, as I lost sight of the patient. 

Another case was that of a girl, sixteen years of age, with right hemiplegia 
and epilepsy. Wyeth performed the first operation on this patient, exposing 
the dura over the entire motor area, and a very large portion of the cortex 
was inspected after the dura had been incised. The exposed cortical tissue 
was tested by the faradic current, and responded normally to mild currents ; 
as the cortex seemed to be normal I did not urge its excision. I regretted at 
that time not having urged the complete excision of the arm centre. I do 
not, however, consider Horsley's reason for removing such centres entirely 
valid, since secondary changes, which have been established, seem, after all, to 
be the main source of irritation, and if one focus of disease be removed I am 
confident that other parts of the hemispheres will soon attain to the dignity 
of epileptogenous centres. It is, furthermore, well established at the present 
day that convulsive seizures may result from irritation of almost any part of 
the cortex. In this case there was very little improvement excepting that the 
epileptic attacks were transferred after the operation to the other half of the 
body, whose centres had not yet been exposed. I have had the other half 
of the brain exposed more recently by Dr. Gerster, but for reasons which I 
have suggested did not urge the excision of any of these parts, and I fear 
that the girl will remain an incurable epileptic. 

A third case was one operated on by Dr. Wyeth. The patient was a 
man, thirty-two years of age, who had had an apopletic attack at the age of 
two years. As a result of this he exhibited a left hemiplegia with considerable 
atrophy, with contractures of the left upper extremities and athetoid move- 
ments of the left hand ; furthermore, pes equinus of the left side with a 



INFANTILE CEREBRAL PALSIES. 46 1 

tendency to equino varus. I made the diagnosis of an old infantile spastic 
hemiplegia, probably due to meningeal hemorrhage over the arm centre in 
the right hemisphere. Dr. Wyeth exposed this centre according to my di- 
rections, and at this point was found a distinct hemorrhagic discoloration of 
the pia, with the development of adhesions between the pia and the cortex. 
I advised that these adhesions be incised, but did not urge removal of the 
cortical tissue, though in this case I -am free to say, since the arm was al- 
ready paralyzed and practically useless, a very liberal excision of this arm 
centre might well have been practised. The man recovered well from the 
operation, and his convulsive seizures have been very much diminished dur- 
ing a period of nearly three years. In October, 1894, Dr. Gerster operated 
upon a fourth case (infantile hemiplegia with epilepsy) in a boy, aged twelve ; 
the epileptic fit began in the left (paralyzed) arm, and then became general. 
The greater portion of the arm centre was excised, showing the effects of old 
hemorrhage (Fig. 145) ; the attacks have not been sensibly diminished. Sim- 
ilar cases have been reported by Weir, Keen, Roswell Park, Angell, and Starr. 
In Weir's case a large cyst was found, which was carefully drained, but the 
child, four years of age, died five hours after the operation. In one of Keen's 
cases the child died twenty minutes after the operation, and the brain exhib- 
ited a very considerable atrophy of the entire left hemisphere. The greater 
danger of all these operations in young children should be taken into ac- 
count, and for this reason I now hesitate to have the operation performed 
upon any child under the age of four years. Wildermuth has reported two 
cases. In the one case he removed atrophic cortical tissue, and evacuated a 
cyst underlying it. Three years after the operation the patient was said to 
have been improved. In another case he also performed excision of an 
atrophic portion of the cortex, and the attacks were said to have been in- 
hibited up to ten months after the operation. 

Dr. Angell, of Rochester, has reported upon a case of infantile hemiple- 
gia (birth case) with imbecility and epilepsy. A simple craniotomy was 
done, exposing a cyst in front of and above the ascending frontal region of the 
left side ; a slight improvement followed. If a cyst is as clearly limited as in 
this case, removal of the cyst, or at least drainage of the same, would have 
been a more rational and radical operation ; the improvement following upon 
craniotomy can be explained as the result of a release of pressure * 

The results of the last few years have been much more encouraging (cases 
of Starr, Chipault, Krause, Oppenheim, Sonnenburg and my own) ; if all the 
symptoms of a case point clearly to an initial focus of disease, surgical pro- 
cedures are quite in order. As cysts are extremely frequent in these infantile 
cerebral palsies, the evacuation and removal of such cysts will often be fol- 
lowed by considerable relief. I believe that it is due to the frequency of such 
cysts that relief follows so many operations for epilepsy. 

If surgical procedures are unavailing, the ordinary anti- 

* While this edition was passing through the press, Cushing reported the success- 
ful removal of large meningeal clots a few days after birth! "The procedure itself, 
though delicate in performance, need not entail serious consequences." 



462 THE NERVOUS DISEASES OF CHILDREN. 

epileptic measures may be employed. Bromides and chlo- 
ral should be given in moderate doses, but there is little 
use in pushing these drugs to the extreme, for we cannot 
expect to cure an epilepsy that is dependent upon an organic 
brain lesion. 

It will be best in this connection to study a disease which 
has evident relations to other family affections (see Chapter 
XXI). Although it is a cerebro-spinal disorder, the author's 
studies on this disease were made largely in connection 
with the study of the infantile cerebral palsies. 

AMAUROTIC FAMILY IDIOCY. 

This name was given by the author to a disease not altogether rare * 
affecting several members of one family, and characterized by an impairment. 
of all the mental functions, by a progressive weakness of some or of all the 
muscles of the body, and by a defect in vision (associated with changes in 
the macula lutea and subsequent optic nerve atrophy), terminating in com- 
plete blindness. The disease is fatal, the children dying, as a rule, in a con- 
dition of complete marasmus before the end of the second year of life. 

History. of the .Disease. — In 1881 Waren Tay described " symmet- 
rical changes in the region of the yellow spot in each eye of an infant." He 
had noted the fact that the child's cerebral development was slow, and also 
that the optic discs were apparently healthy at first, but that, in the region of 
the yellow spot, there, was a conspicuous, tolerably large white spot more or 
less circular in outline, and showing at its centre a brownish-red, fairly circu- 
lar spot contrasting strongly, with the white spot surrounding it. Tay likened 
this " cherry-red spot " to the appearances which are well known in cases of 
embolism of the central artery of the retina, and believed the changes to be 
" possibly congenital." Subsequently he noticed a gradually developing atro- 
phy of the optic discs, while the changes in the macula lutea remained the 
same as before. Tay also reported that three similar cases had occurred in 
this family. These same ophthalmoscopic findings had been noted by a 
number of oculists in Europe and in America. 

So long ago as 1887, without any knowledge of the cases described by the 
oculists, the author reported what appeared to be a peculiar form of idiocy 
associated with blindness. Some years later I became aware of the family 
character of this affection, and it was then shown by Kingdon, of Nottingham, 
that the cases reported by the oculists and those reported by me were iden- 
tical. But it was not until the year 1898, after a much larger experience 
with this category of cases, that I was convinced that this condition deserved 
to be considered as a clinical entity, and in that year I proposed the name 
Amaurotic Family Idiocy, which has been universally adopted. 

* In 1904 Heveroch had collected the records of 86 cases, of which 44 occurred in 
America. 



AMAUROTIC FAMILY IDIOCY. 



463 



Risien Russell has been the only one to use the designation " Infantile 
Cerebral Degeneration. "*' The name is wholly inadequate, and in view of our 
present knowledge of the pathology of the disease, does not do justice even 
to the morbid anatomy of Am- 
aurotic Idiocy. The symptoms 
as described by me have been 
endorsed by all subsequent 
writers. Among these I wish 
especially to mention Peterson, 
Hirsch, Koplik, Kingdon, Rus- 
sell, Falkenheim, Higier, Schaf- 
fer, Fry, Spiller, McKee, Mary 
Buchanan, and Shumway. 

Symptomatology. — The 
children are born healthy (sev- 
eral of them have been unusu- 
ally robust) and do well until 
the age of four, five or six 
months. At about that time 
they become listless, apathetic, 
move their limbs very little, 
and show the first signs of im- 
paired vision. The child is 
unable to hold its head up 
straight or to sit up, and as 
time goes on, instead of gain- 
ing strength in the use of 
its muscles, its limbs become 
limp, in some instances spastic 
and more or less paralyzed. 
The reflexes may remain nor- 
mal, at times they may be a 
trifle subnormal, and in the 
spastic cases they become ex- 
aggerated. An unusual sensitiveness to sound (hyperacusis) is observed at 
a very early date, so that the child is startled by the slightest noise, by a 
clapping of the hands, etc. Oppenheim speaks of this symptom as an in- 
creased motor acoustic reflex. Convulsions may occur, but are not an integral 
feature of the disease. After the lapse of a few months the mental impair- 
ment is distinctly increased and blindness becomes absolute, and the child 
lapses into a condition of extreme marasmus, in which it dies, as a rule, be- 
fore the end of the second year (Fig. 1 15a). 

We may sum up the chief symptoms as follows: (1) A mental impair- 
ment observed during the earlier months of life and leading to absolute idiocy. 
(2) A paresis or paralysis of the greater part of the body which may be either 
flaccid or spastic. (3) The reflexes may be normal, deficient, or increased. 
(4) A diminution of vision terminating in absolute blindness (cherry-red spot 




Fig. 115. — Case of Amaurotic Family Idiocy, 14 
months old. Patient of Dr. Cotton, of Chicago, 
showing well-nourished condition of child in 
earlier stage of disease. 



464 



THE NERVOUS DISEASES OF CHILDREN. 



in the region of the macula lutea with subsequent optic nerve atrophy). 
(5) Marasmus, and a fatal termination, as a rule, at about the age of two 
years. (6) Unusual sensitiveness to sound (hyperacusis). (7) The appear- 
ance of the affection in several members of the same family. 

In some of the children I have noted nystagmus and strabismus. A 
few other symptoms in addition to those described above have been men- 
tioned by Falkenheim. These are "explosive laughter" and "disturbances 
of deglutition." The former I have not observed, but it is very probable that 
it may occur in some of the cases, and the latter may be taken to be an 
accompaniment of the great mental and physical deterioration ; and the occur- 
rence of such bulbar symptoms need not 
astonish one, in view of our present 
knowledge of the anatomy of the disease. 
The ocular symptoms constitute a 
most striking feature of the disease. In 
every instance in which the opportunity 
for ophthalmoscopic examination has 
been given, this peculiar condition was 
found present, but I wish to state dis- 
tinctly that the disease can be diagnosti- 
cated, as has been done, before the oc- 
currence of the typical changes in the 
region of the macula lutea. In Hig- 
ier's case optic nerve atrophy was much 
more pronounced than the changes in 
the macula lutea. All writers on the 
subject have fully appreciated the char- 
acteristic symptoms of Amaurotic Idiocy, 
but the importance of the rapidly devel- 
oping marasmus -has not been sufficiently 
insisted upon by many of them. I hold 
it to be one of the important symptoms 
of the condition. 

Etiology. — The causes underlying 
this disease are still somewhat obscure. 
Blood relationship between the parents 
has been noted in some instances and a 
marked psycho-neurotic taint in others. 
In a few instances the mother has sus- 
tained some injury during pregnancy. 
The family predisposition is made evi- 
dent by the fact that twenty-eight cases 
which came under my notice have oc- 
curred in fifteen families. Carter was the 
first to call attention to the fact that all cases reported have occurred among 
Hebrews, and up to the present time not a single entirely satisfactory case 
has been reported as occurring among other races. It is truly astounding 




Fig. 115a. — Child in Last Stages of 
Amaurotic Family Idiocy, showing 
extreme emaciation and (unusual) 
contractures. Author's case. 



AMAUROTIC FAMILY IDIOCY. 



465 



that this disease should be thus limited, because other diseases to which it is 
more or less closely allied have been observed among all races and all 
nationalities. 

It is well to state explicitly that this is not a syphilitic disease, and this 
point should be insisted upon, because hereditary optic nerve atrophy occur- 
ring later in life has been shown to be due frequently to hereditary syphilis, 
and there are other family affections associated with dementia which are due 
to this same cause. 

Diagnosis. — There can be little difficulty in diagnosticating this form of 
disease if the symptoms as enumerated above are kept in mind. Hereditary 
spastic diplegia, which sometimes occurs in families, may give rise to some 
confusion, but in this disease the entire absence of visual symptoms, the lack 
of mental impairment, the marked spasticity of all limbs, and the fact that the 
children show no tendency to marasmus will help to bring out the true diag- 
nosis. Hereditary optic atrophy may also be confounded with Amaurotic 
Idiocy, but the former only occurs in older persons. Several times the author 
has been asked to see cases which were supposed to be instances of Amau- 
rotic Family Idiocy which presented the symptoms due to a preceding men- 
ingitis ; and on the other hand, the author is also aware that in not a few 
instances the diagnosis of meningitis has been made when the case was a 
very typical one of Amaurotic Family Idiocy. Rayner D. Batten has re- 
ported symmetrical disease of the macula lutea in two brothers beginning at 
the age of 14 years, and F. E. Batten describes "cerebral degeneration" 
with symmetrical changes in the macula lutea in two members of a family. 
These cases are, however, very distinct from the type here described. In 
Rayner D. Batten's cases there was hereditary syphilis ; yet it is of interest to 
note the family occurrence of ocular changes in and around the macula lutea. 




Pathological Anatomy. — In three brains which the author has had 
an opportunity of examining there were peculiar abnormalities of fissura- 



466 



THE NERVOUS DISEASES OF CHILDREN. 



tion (confluence of the fissure of Rolando and fissure of Sylvius and in two 
instances exposure of the island of Reil). Such abnormalities are generally 
conceded to be a sign of deficient cerebral development (Fig. 116). In the 
earlier publications on this subject I spoke of the morbid condition as an 
Agenesis corticalis, a defect in the development of the highest nerve elements 
of the cortex. There was also some degeneration of the white fibres in the 
hemispheres and of the pyramidal tracts in the spinal cord. This degeneration 
of the white fibres could be traced through the course of the pyramidal tracts 
in the internal capsule, crusta, pons and medulla. But more striking than 




Fig. 117. — Section through XII nucleus in medulla oblongata, showing advanced 
changes in ganglion cells. Hematoxylin-eosin stain. 



this change in the white fibres is the change in the gray matter not only of 
the cortex, but throughout the entire central nervous system. The changes 
are found to be the same in the cortex of the brain, in the cranial nerve nu- 
clei, and in the anterior and posterior gray matter from the cervical to the 
lumbar and sacral segments of the cord. Even the spinal ganglia showed 
similar changes. Our knowledge of the spinal changes we owe chiefly to 
Dr. Hirsch, whose findings I have been able to corroborate in a case exam- 
ined by me only a few years ago. There can no longer be any doubt that 
the disease is due to a degeneration of the ganglion cells throughout the en- 
tire central nervous system. In the cortex, as well as in the gray matter of 



AMAUROTIC FAMILY IDIOCY. 



467 



the cord, there is scarcely a normal ganglion cell to be seen. As will be 
noted from the illustrations herewith presented (see Figs. 117-119), the 



>w 




Fig. 118. — Section through cervical enlargement, showing cell changes in anterior 
gray matter. (Van Gieson stain.) 

cell-body is completely altered; the entire cell protoplasm has become disin- 
tegrated, leaving a more or less homogeneous mass; the nucleus has been 




^@^3#^€>_ k 



Fig. 119.— Section through a lumbar ganglion. Nissl stain showing marked cell 

changes. 

shifted commonly to some part of the periphery of the cell, and instead of 
being distinctly differentiated from the rest of the cell-body, it generally 



468 THE NERVOUS DISEASES OF CHILDREN. 

shades off by degrees into the disintegrated structure of the cell. The nu- 
cleus is often wanting entirely, and in some instances the whole nucleus ap- 
pears to have dropped out. The disintegration of the cell-body is so complete 
that in many specimens the contour alone enables one to infer that these 
round bodies are the remnants of ganglionic cells. These metamorphosed 
ganglion cells are frequently surrounded by distinctly pericellular spaces, and 
are relatively and absolutely so much enlarged that they give a very striking 
appearance to the cross-section. 

Holden, as well as Shumway and Mary Buchanan, have made careful his- 
tological examination of the eyes in Amaurotic Family Idiocy. All these 
writers are agreed that the essential changes in the eyes are " a degeneration 
of the ganglion -cells of the retina and of the nerve-fibres of the optic nerves 
and tracts, which are genetically a portion of the central nervous system." 
They also believe that the white area in the fundus is the result " of the 
swollen and degenerated ganglion-cells which are present in much greater 
numbers in the macula region than elsewhere," and do not consider this white 
area to be due to an oedema of the tissue as was first supposed. 

Hirsch, Spiller, Shaffer, of Budapest, and the author have proved that Am- 
aurotic Family Idiocy is due to a degeneration of the entire gray matter of the 
brain and spinal cord. The degeneration of the white fibres of the anterior 
and lateral pyramidal tracts is in all probability secondary, but not nearly so 
marked as one would expect with such advanced disease of the ganglionic 
elements. It is probable that the relative involvement of the gray and white 
matter may vary much in different subjects affected with this disease. 

Hirsch and some others have supposed that Amaurotic Family Idiocy was 
an acquired disease; that the morbid changes in the cells were of a distinctly 
degenerative character, and that these changes were similar to those produced 
by toxic agents of various sorts. Hirsch went to the extent of claiming that 
the toxic agent might possibly be conveyed to the child through the mother's 
milk. It is difficult to explain any family disease on the supposition that it is 
of toxic origin, and in this instance the author must repudiate this view for the 
simple reason that several of his patients have not been nursed by their own 
mother; and it is very evident that, although the symptoms of the disease are 
not always noticed before the age of three or four months, the development 
of the disease is due to conditions antedating the time of birth. The author 
still firmly believes that Amauratic Family Idiocy is due to an arrest of de- 
velopment, and that this arrest of development is followed by a degeneration. 
Accepting Gowers' and Edinger's views of the inherent or deficient capacity 
for normal development, we may simply argue that children who are afflicted 
with Amaurotic Family Idiocy have a very limited capacity for normal de- 
velopment ; that their central nervous system is not equal to the functions that 
they are expected to perform for more than the first three or four months of 
life, and from that time on .a rapid deterioration of all functions is established. 

Prognosis. — The disease is invariably fatal; between the age of twelve 
and eighteen months the children, even though they have been in tolerably 
robust health at birth, pass into a condition of extreme marasmus. Only one 



HEREDITARY SPASTIC PALSIES. 469 

case in which the diagnosis of Amaurotic Family Idiocy could be accepted 
has lived beyond the age of two years. 

Treatment. — No form of treatment has been of any avail. It is very 
positive that the disease is not due to hereditary syphilis, and for that reason 
even the ordinary iodide treatment cannot be urged. In several families in 
which children with this disease have been observed, others have been born 
and have remained entirely healthy. No further warning should therefore be 
given to parents of such children ; let them, however, exercise special care 
with reference to the physical development and early training of their off- 
spring. 

Freud has reported several interesting cases under the title of " Family 
Forms of Cerebral Diplegia." Two of the children were bright at the ages 
of six and five years respectively, but they presented from earliest childhood 
the following symptoms : Nystagmus, atrophy of the optic nerve, convergent 
strabismus, awkwardness in speech and in the use of the arms ; tremor of 
the arms and a spastic paraplegia of the lower extremities ; no convulsions. 
In these boys the disease is evidently present in its lightest and most favor- 
able form. The improvement in vision as the one child grew older seems 
evidence of this, but the resemblance of these forms to those described by 
me is shown by a third child that died at the age of ten months ; it was 
paralyzed from birth, and idiotic ; whether it was blind also is not stated. The 
father thought the child had died of rickets (more probably of marasmus). 

Pelizaeus has described a family disease of which the chief symptoms 
were : Nystagmus, mental imbecility, disturbances of speech (Bradylalia), 
awkwardness of hands, spastic paraplegia of the lower extremities. The 
disease attacked male members of several generations, but was transmitted 
through healthy mothers. All of those who were attacked showed some 
symptoms in childhood, and several died young. Pelizaeus was inclined to 
the diagnosis of multiple sclerosis ; but his cases evidently constitute a late 
form of hereditary spastic paralysis. 



CHAPTER XXVI. 

TUMORS OF THE BRAIN AND ITS MENINGES. 

New-growths within the skull are very common in 
childhood. There is no period of early life that is entirely 
exempt from them. Careful studies of cerebral tumors 
have been made by Steffen, Bernhardt, Starr, Knapp, Bruns, 
and Oppenheim. The following tables are taken from an 
article by Peterson : 

Table I. 

Form of Tumor. No. of Cases. 

Tubercle 166 

Glioma 42 

Sarcoma 37 

Cyst 35 

Carcinoma n 

Gliosarcoma 5 

Angiosarcoma 1 

Myxosarcoma 1 

Papillary epithelioma 1 

Gumma 1 

Not stated 35 

Total 335 

Table II. 

Site of Tumor. No. of Cases. 

Cerebellum 105 

Pons Varolii 42 

Centrum ovale 41 

Basal ganglia and lateral ventricles 30 

Corpora quadrigemina and crura cerebri 25 

Cortex cerebri 23 

Medulla oblongata 7 

Fourth ventricle 6 

Base of brain 8 

Total 287 



TUMORS OF THE BRAIN AND ITS MENINGES. 471 

From these tables we can infer, first of all, that tubercle is much more 
frequent than any other form of intra- cranial neoplasm, very nearly one-half 
of all the cases being of this character. Glioma and sarcoma are the next 
most common forms, and as the cerebral tissue in earlier years is subject to 
proliferation, the frequent occurrence of the former need not be a matter of 
surprise. Cysts differ from tumor in this respect that they are secondary 
products of some preceding morbid process, very often resulting from an old 
hemorrhage, or from embolic softening. The contents of a cyst may remain 
stationary for a very long time, and under such circumstances the symptoms 
will not be very like those of a growing tumor. In other instances the con- 
tents of the cyst suddenly increase and all the symptoms may be those of a 
rapidly increasing neoplasm. 

As for the location of tumors in general, it will be seen that a larger 
number are found in the cerebellum than in any other part of the brain. 
The pons and the centrum ovale are next in order of frequency, then come 
the basal ganglia, and last the cortex of the brain, and the region of the cor- 
pora quadrigemina. The medulla oblongata and the base of the brain are 
rarely the seat of tumor. 

Etiology. — The actual cause of cerebral tumors in 
children is a matter of considerable speculation. It is only 
with regard to gliomata that we can state that the natural 
tendency of the infantile brain to proliferation of its tissue 
upon the slightest provocation accounts for the frequent 
occurrence of this special form of neoplasm. Tubercle, 
sarcoma, and carcinoma of the brain, are always secondary 
to deposits of a similar character in other parts of the body. 
Carcinoma is rare in children, and carcinomatous deposits 
in the brain are correspondingly infrequent, but under excep- 
tional conditions these may occur early in life. A few cases 
have been observed which were due to an invasion of the 
growth from the orbit. The exact relation of external in- 
juries to new-growths has been often in dispute. It is more 
than probable that the accident to which the tumor is re- 
ferred is, as a rule, merely a landmark in the child's 
history. 

In the fewest instances only can an actual causal relation between the two 
be established. The hyperasmic condition or the slight hemorrhages which 
occur at the seat of injury may at times be sufficient to become the starting- 
point of a latent morbid process. Boys are more liable to brain tumors than 
girls, and those who are not too careful in utilizing statistical evidence will 
claim that this fact is to be explained by the greater frequency of injuries to 
the boys' skulls. 



47 2 THE NERVOUS DISEASES OF CHILDREN. 

Symptoms. — As in the adult, so in the child, the symp. 
toms of intra-cranial tumor may be divided into two distinct 
groups. The first includes the general symptoms resulting 
from increased pressure, and the second group includes 
those due entirely to the location of the tumor. It is a 
matter of course that slowly progressive tumors will exhib- 
it less symptoms than those which invade the brain rapidly 
and soon attain to a very considerable size. A tumor of 
small size growing rapidly, will produce far more symp- 
toms than one of huge size which has taken years to de- 
velop. The symptoms will also vary according to the 
blood-supply of the tumor, and according to the increase 
in the amount of hydrocephalic fluid. The general symp- 
toms which we must consider somewhat in detail are head- 
ache, nausea, vomiting, stupor, insomnia, convulsions, slow 
pulse, and double optic neuritis. 

Headache is in many cases the most striking symptom of tumor of the 
brain. The nearer the tumor is to the cortex, and the more it involves the 
meninges, the more intense on the whole the headaches are likely to be ; but 
I have seen headaches as intense as those of cortical tumors in cases in which 
the growth was in the vicinity of the ganglia, or even at the base of the brain. 
In such patients the increase in cerebro-spinal fluid has much to do, in all 
probability, with the development of the headaches. The intense " boring," 
" gnawing " pain is generally referred to that part of the head which is nearest 
to the new-growth ; but there are exceptions to this rule as to every other. 
In children whose fontanelles are not completely closed the tumors may 
attain to a considerable size without causing intense headaches. These 
headaches are often the cause of sleeplessness, and both the pain and the 
insomnia are largely responsible for the rapid emaciation of the child. In 
some few instances insomnia is present without pain, and is the result of 
cerebral excitement. 

The association of nausea and vomiting with headaches in a child should 
lead to the suspicion of tumor, particularly if all these symptoms persist for 
some considerable period of time. The vomiting is characterized by its sud- 
denness, and by its projectile character. Nausea may be associated with it, 
but in many cases there is no warning given of the vomiting, and it comes as 
a surprise upon the child, and often occurs entirely independently of meals ; 
at other times during meals. Vertigo is not infrequent in cases of tumor, and 
is very apt to occur with every change in position of the head. It is very fre- 
quent indeed in connection with tumors of the cerebellum, or of the pons, 
which is explained by the relation of these parts to the auditory nerve. 

Convulsions in children are so common that they alone need not give rise 
to the suspicion of tumor, but if associated with constant headaches, with 



TUMORS Ofi THE BRAIN AND ITS MENINGES. 473 

vomiting, and if all these symptoms are frequently repeated, the suspicion may 
be well founded. These convulsions are very often general, but in addition 
to the general convulsions, or without the occurrence of such, localized con- 
vulsions may occur if the neoplasm is in some part of the motor area. Such 
localized convulsions, associated with headaches, vomiting, and with optic 
neuritis, point very definitely to an intra-cranial growth. 

Double optic neuritis and the complete optic atrophy 
following it are important symptoms of intra-cranial tu- 
mor. It is certain that there is no symptom which if pres- 
ent, is more valuable than an optic neuritis ; but, on the 
other hand, tumors may attain considerable size without 
the development of optic neuritis. This neuritis is in all 
probability due to increase of intra-cranial pressure. Other 
theories which have been proposed do not explain all the 
peculiarities of optic neuritis as well as the theory of in- 
creased pressure does. The fundus should be examined in 
every doubtful case of brain tumor, for normal vision is 
not incompatible with a considerable degree of optic neu- 
ritis, and changes in the optic nerve may occur, and do oc- 
cur, with tumor in any part of the brain ; but they occur at 
a much earlier period and to a much more intense degree 
with tumors situated at the base of the brain. Under these 
conditions the direct pressure upon the optic chiasm and 
the greater increase of intra-cranial fluid at the base may 
account for the earlier appearance of the symptoms. The 
optic neuritis is generally double, though it may be more 
distinct for a time in one eye than in another. 

Sudden variations in the pulse-rate, and in rapidity of res- 
piration have been observed ; Cheyne-Stokes respiration I 
have seen in children suffering from cerebellar tumors long 
before the terminal stage had been reached. 

The presence of tumor can often be suspected from the 
occurrence of these general symptoms. The signal or 
localizing symptoms are dependent upon the special func- 
tions of the areas invaded by the neoplasm. These localiz- 
ing symptoms are the best illustrations in man of cerebral 
functions, as they have been demonstrated by experiments 
upon animals. The matter is not quite as simple, however, 
as it would seem, for a neoplasm causes both direct and in- 
direct symptoms. The direct symptoms are those caused 



474 THE NERVOUS DISEASES OF CHILDREN. 

by the actual loss of function due to displacement of tissue 
by the neoplasm. The indirect symptoms are caused either 
by the distant effects of the pressure by the tumor upon 
neighboring- parts, or they may result from the secondary 
increase of intra-cranial fluid. At all events, the symptoms 
first produced are the most important ones, and will be of 
the greatest value in determining the exact site of the 
lesion. 



The indirect symptoms are perhaps more frequent in the cases of cere- 
bellar tumors than in any other. Thus in this form we may have the gen- 
eral symptoms of intra-cranial neoplasm, together with reeling and distinct 
occipital headaches. Associated with these we often find a paralysis of the 
rectus externus supplied by the sixth nerve. Spinal symptoms may occur 
with cerebellar and other intra-cranial growths. Batten and Collier, Anton, 
Pick, and a number of German writers have referred to the frequent involve- 
ment of spinal cord structures (the posterior roots and posterior columns), 
evidently due to the increased pressure of the cerebro-spinal fluid. 

Macewen's Symptom. — " The elicitation of a differential cranial per- 
cussion-note as an aid to cerebral diagnosis in certain gross changes of the 
intra-cranial contents, especially in children," has been practised by Macewen 
for many years and described in his monograph on " Infective Diseases of the 
Brain and Spinal Cord." For the present, the symptom is most valuable in 
the case of abscesses and of over-distended lateral ventricles. In cases of 
tumor near the surface of the brain the percussion-note will prove of service. 
I have noted a dulness in several cases of tumor of the brain (one case in 
the adult and two in children) ; if the lateral ventricles are over-distended 
the resonance is greatly increased. But distention of the ventricles may be 
secondary to tumor, and in this way increased resonance of the percussion- 
note may be significant. The percussion-note of normal crania seems to vary 
much ; it will be best, therefore, to use this symptom very cautiously. The 
note can be elicited best by tapping the skull lightly with the finger or 
percussion-hammer, and receiving the note through a stethoscope placed 
upon the pterion, or upon the middle of the forehead. 

Tumors of the Cortex. — It is not an easy matter to distinguish be- 
tween the purely cortical tumors and those situated in the subjacent white 
matter. Some claim that it is impossible to differentiate between cortical 
and subcortical tumors, but this seems to me to be rather overstating the 
facts. While the localizing symptoms may be exactly the same in both 
these classes of tumors, the order of development of the symptoms will 
be different, and may give some indication of the exact site of the tumor. 
In subcortical tumors the march of the symptoms is generally such that 
the approach of the lesion toward the cortex is marked by a special series 
of symptoms. Taking, for example, the tumors in the motor area, those 
in or near the gray matter, however small, are apt to give rise to occa- 



TUMORS OF THE BRAIN AND ITS MENINGES. 47 S 

sional convulsive seizures from the very start ; whereas, in the tumors which 
begin in the subjacent white matter, the paralytic symptoms may appear at 
a very early day and may exist for some time before any symptoms of corti- 
cal irritation arise. Thus, in a case under my observation, the patient had 
gradually developed paralysis of the arm, of the face, and slight paresis of 
the leg on one side of the body. He had indistinct headaches and optic 
neuritis, and yet, during the first three months after the onset of these symp- 
toms, not a single convulsive twitching occurred. Since that period epilep- 
toid seizures have set in, and the occurrence has proved that the tumor has 
finally invaded the cortical region. The invasion of the cortex, moreover, is 
more likely to lead to repeated convulsive seizures and to increased head- 
aches from the more direct involvement of the meningeal coverings ; the 
nearer the surface and the nearer the meninges the more intense these head- 
aches become.* Certain classes of tumors, moreover, are more apt to begin 
in the cortical tissue than in the subcortical white substance ; thus tubercles, 
gliomata, and gummata, above all, are almost certain to begin near the sur- 
face, while sarcomata and cysts are as often subcortical as cortical in origin. 

Tumors of the Frontal Lobe. — Since the frontal lobe has no dis- 
tinct and special function, tumors in this region are often developed without 
localizing symptoms. If the tumor extends downward and involves the ol- 
factory bulb the sense of smell may be diminished or lost ; but changes in 
character and intelligence, " punning," irritability or stupidity, are considered 
to be relatively frequent in diseases of the frontal lobe, and this is entirely in 
support of the conditions which Goltz first discovered as a change in the be- 
havior of dogs whose frontal lobes were excised or destroyed. More recently 
Torje has recorded a case in which there was marked disturbance of saliva- 
tion ; he suggests that the drooling in idiots may be due to defective frontal 
development. Bruns has mentioned the occurrence of cerebellar ataxia in 
these cases. Frontal tumor may also be diagnosticated if the general symp- 
toms were present for a long period of time, and then symptoms pointing to 
the third frontal gyrus or the motor areas appear as evidence of the gradual 
encroachment of the new-growth upon these areas. 

Tumors of the Third Frontal Convolution. — The third frontal 
convolution is the seat of motor speech function, and any destructive lesion 
in or near this part of the brain will cause typical motor aphasia. Agraphia 
may be associated with motor aphasia. The cases in which motor aphasia, 
or agraphia, or both, have signalized the invasion of a tumor are compara- 
tively rare, particularly in children ; for if the invasion of the tumor be a grad- 
ual one the right hemisphere may, in right-handed persons, gradually assume 
the functions of speech, which up to that time have been inherent in the left 
hemisphere. In addition to this, in young children the differentiation between 
the left and the right hemispheres is not nearly as complete as in later years. 

Tumors of the Motor Area. — These can be recognized more readily 
than those in any other part of the cortex. In the earlier stages of the dis- 

* If there is excessive intra-cranial pressure, headaches may be intense, even in 
tumors distant from the surface. 



476 THE NERVOUS DISEASES OF CHILDREN. 

ease symptoms of cortical irritation are present, and convulsive twitchings of 
the paretic or paralyzed part will indicate the probable site of the tumor. 
Thus, if a patient with slight sensory and motor disturbances in the upper 
extremity should experience occasional convulsive twitchings of the arm, of 
the forearm, or even of the thumb alone, the character and extent of these 
twitchings would point to the part of the brain first and chiefly involved. 
According to the course and development of the convulsive seizures the 
direction in which the growth extends can be judged. Vasomotor symptoms 
and changes in pulse-rate are uncommon. 

Tumors of the Parietal Lobe. — Tumors of the parietal lobe may 
cause no distinct localizing symptoms, but the records of Mills and Spiller 
prove conclusively that lesions in these parts are apt to be followed by sen- 
sory changes (loss of muscular sense, astereognosis, etc.). These facts of 
human pathology are in keeping with the results of Munk's experiments on 
dogs, and those of Schafer and Horsley on monkeys. These sensory symp- 
toms, however, are not so constant that their absence would militate against 
the diagnosis of tumor in this region. If a tumor in the parietal lobe affects 
the subjacent white matter it may result in hemianopsia, for the white tract 
of Gratiolet passing from the internal capsule to the occipital lobe lies under 
this portion of the cortex. Ferrier's claim, that the centre of vision lies in 
the angular gyrus, is due to the involvement by disease in this region of the 
optic radiations which we have mentioned. By extension to the inferior 
parietal lobule the tumor may give rise to that peculiar disturbance of speech 
known as word-blindness. The patient cannot read and write at will, though 
he may be able to write upon dictation, or to copy written signs. In children, 
excepting those of a more advanced age, cases will rarely arise in which 
word-blindness or word-deafness are important symptoms. According to 
Wernicke the conjugate movements of the eyes are governed by a centre in 
the inferior parietal lobule. If there is distinct impairment of this one func- 
tion, disease in this region may therefore be suspected. 

Tumors of the Occipital Lobe. — Tumors in the occipital lobe are 
recognized by the peculiar disturbances of vision. As each occipital lobe is 
connected with one-half of each eye, tumor in this region is signalized by 
homonymous hemianopsia without any other special symptoms ; convulsions 
may occur, and not necessarily on account of the direct extension of the 
lesion into the motor areas, but because, as has been shown by Binswanger, 
disease in any part of the cortex may cause epileptic disturbances. The 
researches of Notbnagel and Seguin, which have been corroborated by other 
authors, points with a great degree of certainty to the cuneus as the actual 
centre of vision. Visual hallucinations and word-blindness have been re- 
corded by a number of reputable writers. According to Bruns, optic neuritis 
is relatively infrequent. 

Tumors of the Temporo-sphenoidal Lobe. — The temporo-sphe- 
noidal lobe contains the centres for hearing and for sensory speech ; it is, 
therefore, natural to expect that in the case of tumor in these auditory re- 
gions hearing will be impaired, not abolished, on the side opposite the lesion, 
and sensory aphasia will be present. The patient will be able to speak spon- 



TUMORS OF THE BRAIN AND ITS MENINGES. A77 

taneously and correctly, but will not be able to understand what is being 
said to him, and will, of course, not be able to repeat spoken language. The 
differential diagnosis between tumor and abscess should be established care- 
fully. Deep-seated tumors may cause hemianaesthesia, hemiplegia, and 
hemianopsia. 

Tumors of the Basal Ganglia. — Tumors of the basal ganglia and the 
adjacent parts are not rare. The symptoms which they produce are, as far 
as we know, entirely due to direct or indirect involvement of the internal cap- 
sule. As this capsule contains the entire motor tract for the opposite side of 
the body, the sensory fibres, the fibres of special sense, the speech-tracts, we 
can infer the multiplicity of symptoms which may result from such a le- 
sion. The position of these fibres in the internal capsule is quite well es- 
tablished, and in the case of small tumors, the order of involvement of the 
different functions may give some clew as to the direction in which the tumor 
is extending. The tumor may impinge, too, upon the lateral ventricles, and 
thus cause considerable disturbance. Some of the symptoms may be sec- 
ondary to the increase of hydrocephalic fluid. On account of the compact 
structure of the inner part of the brain, even small tumors will cause con- 
siderable distortion of the parts, and in addition to all the other symptoms 
presented as the result of the involvement of the internal capsule, we may 
have symptoms resulting indirectly from pressure upon the cranial nerves, 
which come off from the brain axis below this region. The differential diagno- 
sis between tumors of the ganglia and tumors of the cortex will be based in 
part upon the absence of convulsive seizures in the case of ganglionic tu- 
mors, and the more frequent presence of choreic or athetoid movements 
with cortical disease. 

Tumors of the Crus Cerebri. — A neoplasm in this region can be 
recognized very early by the association of oculo-motor symptoms with paral- 
ysis of motion and sensation of the opposite half of the body. The eye 
will exhibit ptosis, paralysis of all the external muscles except the rectus ex- 
ternus and the superior oblique, and complete paralysis of the sphincter 
iridis, and the ciliary muscle. 

Both peduncles are so close to one another that a tumor occurring in one 
may actually invade the opposite side, or at least press against the peduncle 
of the other half of the brain. For this reason it is not uncommon to find 
paralysis of both halves of the body, or possibly double ptosis and double 
oculo-motor symptoms in tumors in this region. In the majority of cases, 
however, the symptoms are strictly unilateral for a long period, and become 
bilateral later on. 

Tumors of the Corpora Quadrigemina. — These tumors should 
in reality be considered with tumors of the peduncles, for these structures are 
so closely related to one another, anatomically and physiologically, that the 
occurrence of neoplasm in the one part or the other will produce a common 
series of symptoms. It is only the occurrence of oculo-motor paralysis with 
opposite hemiplegia that suggests the region of the peduncles, while a few 
additional symptoms refer distinctly to the corpora quadrigemina. These 
additional symptoms are due to the relation which the corpora quadrigemina 



478 



THE NERVOUS DISEASES OF CHILDREN. 



bears to visual functions, and to the connection between the former and the 
cerebellum. Loss of pupillary reflexes, nystagmus, vertigo, and a condition 
resembling cerebellar ataxia, point to the region of the corpora quadrigemina 
as the special seat of the lesion. I had occasion a few years ago to publish 
an interesting case of this description, with the result of the post-mortem 
examination (Fig. 120). Tumors of the pineal gland will cause an entirely 
similar series of symptoms. Marked vasomotor disturbances have been 
recorded by Collins and others with neoplasm in this region. 

Tumors of the Pons. — Tumors of the pons and medulla, like other le- 
sions in these parts, give rise to a great multiplicity of symptoms. The pyram- 
idal tracts are closely approx- 
imated to one another near the 
median line, so that, though the 
symptoms may be unilateral, 
they are often bilateral. In ad- 
dition, therefore, to the hemiple- 
gia, or double hemiplegia, we 
may have other symptoms point- 
ing to an involvement of the 
various cranial nerves. A neo- 
plasm in the upper half of the 
pons may encroach more upon 
the peduncles than upon the 
structures of the pons itself, re- 
sulting in a hemiplegia of one 
side of the body with an in- 
volvement of the third and fifth 
nerves of the opposite side. If 
the tumor is in the lower half 
of the pons, the fifth, sixth, sev- 
enth, and eighth nerves will be 
more or less involved, and the 
symptoms resulting from this 
affection will be paralysis of the 
rectus externus, paralysis of all 
the branches of the seventh nerve 
in one half of the face, and a loss 

of hearing in one ear. All these cranial nerve symptoms will be on the side of 
the lesion opposite the hemiplegia. If the sixth nerve nucleus is affected there 
will be, in addition, distinct paralysis of one rectus externus muscle, and pa- 
ralysis of the conjugated movements of the eyes toward the side of the lesion, 
for this nucleus is connected with the third nerve nucleus of the opposite side 
and governs the outward movement of each eye. In spite of this conjugate 
paralysis each internal rectus if examined separately may exhibit normal 
movements. If the lesion is near the surface of the pons, and away from the 
nucleus, it will involve the root of the sixth nerve, and will cause paralysis of 
the rectus externus muscle of one side, but will not affect the conjugate 




Fig. 120. — Section passing through the Pos- 
terior Quadrigeminal Bodies. T, tumor (sol- 
itary tubercle) ; P. Q, posterior quadrigemi- 
nal body. 



TUMORS OF THE BRAIN AND ITS MENINGES. 479 

movements of the opposite side. If the patient is directed to look toward 
the side of the paralyzed rectus or toward the side of the tumor the opposite 
eye will move promptly, the affected eye remaining fixed. This differentia- 
tion between the isolated paralysis of the rectus externus and paralysis of the 
conjugated movements is the most valuable, and perhaps the only differen- 
tial point of diagnosis that helps at times to distinguish between a tumor 
near the surface and one within the substance of the pons. 

The difficulties of diagnosis of pons lesions are increased by the exceeding 
variability of the symptoms, and by the fact that some of the nerves in the 
pons escape disease, whereas others may be intensely affected. 

In the case of a tumor in the medulla oblongata the symptoms are very 
similar to those met with in bulbar palsy. The symptoms will indicate dis- 
ease of the glosso-pharyngeal, of the vagus, of the spinal accessory, and of 
the hypoglossal nerves, together with a unilateral or bilateral paralysis of the 
arms or legs ; the facial nerve will not be involved. Difficulties in degluti- 
tion, in respiratory and cardiac movements ; paralysis or spasm of the sterno- 
cleido mastoid, and of the trapezius ; paralysis and atrophy of the tongue, 
together with vomiting, with glycosuria or polyuria — all these signs will 
suggest a lesion in the medulla. Tumors in this region, and particularly 
gummata, are not rare. Difficulties in diagnosis often arise on account of 
the bilateral character of all the symptoms, but it should be remembered that 
the two halves of the brain at this level are scarcely separated from one an- 
other, and that it is natural for all but vascular lesions (and even these may 
do so) to produce bilateral symptoms. In the pons and medulla the sensory 
tracts also lie very closely to the motor, and for this reason the symptoms 
may be still further complicated by the occurrence of partial, or complete, or 
double hemianaesthesia. As the cerebellum is also in close juxtaposition to 
the medulla and the pons, symptoms of cerebellar involvement may be pres- 
ent in the cases of neoplasms, both in the pons and in the medulla. Optic 
neuritis is developed at a very early day, and occipital headaches are par- 
ticularly severe. 

Tumors of the Cerebellum. — Tumors of the cerebellum are the 
most frequent. The symptoms are occipital or frontal headache, projectile 
vomiting, rigidity of the neck, and (at a relatively early period) a peculiar 
staggering or reeling gait (cerebellar titubation). Double optic neuritis has 
been found in eighty-nine per cent, of these cases. Inco-ordination is 
frequent. Asthenia or muscular weakness is a real cerebellar symptom 
(Mills). Asynergia or hemiasynergia (Babinski) — asynchronous movement of 
the thigh and leg in extending the limb — is of questionable value. Vertigo is 
also more frequent and more severe in cerebellar tumors than in many others. 
In my own rather extensive experience I have found that the patient is apt to 
fall toward the side on which the tumor is located. Mills believes that destruc- 
tive and irritative lesions of the vermis are responsible for a tendency to fall 
forward or backward. A cerebellar tumor may be of very small size and yet 
sufficiently large to compress the sixth, seventh, and eighth nerves, and in its 
further growth may produce involvement of the bulbar nerves as well, just as 
tumor of the bulb may also produce cerebellar symptoms. The affection of 



480 



THE NERVOUS DISEASES OF CHILDREN 



the sixth nerve, causing paralysis of the rectus externus, is extremely common 
in cerebellar tumors, and in some cases the diagnosis of cerebellar tumor may 
be corroborated by the early involvement of the seventh and eighth nerves 
(Fig. 121). 

Of late years, Monakow, Sternberg, Oppenheim, Fraenkel, and Hunt have 
called attention to slowly growing tumors (neuromata and neurofibromata) 
in the cerebello-pontile angle, involving one or more cranial nerves, especially 
both auditory nerves. Cerebellar symptoms are preceded, often for many 
years, by those pointing to involvement of the auditory, the trigeminal, the 




Fig. 121. — Tumor (Sarcoma) of the Cerebello- Pontile Angle. Early appearance 
of facial and auditory nerve symptoms. 

vagus, of one or both sides. I have under observation at the present time a 
young girl who, for years past, has suffered from central deafness, ataxia of 
the right upper extremity, dizziness, and who now is also completely blind 
from double optic neuritis. 

The general manifestations of cerebellar tumors do not vary much from 
tumors in other parts of the brain ; but if the growth encroaches upon the 
pyramidal tracts in the pons and medulla unilateral or bilateral paralysis will 
follow. The reflexes are sometimes exaggerated, as in all other cerebral 
diseases, but in not a few cases the knee-jerks are either diminished or 
absent. 

Differential Diagnosis. — The diagnosis between tu- 
mor and abscess will depend upon the frequent presence cf 
fever, and upon the very slow invasion and slow develop- 
ment of all the symptoms in the latter condition, also upon 
the occasional absence of optic neuritis in spite of the 
presence of symptoms pointing to increased intra-cranial 






TUMORS OF THE BRAIN AND ITS MENINGES. 48 1 

pressure; but the differential diagnosis will depend first and 
foremost upon the proof of the presence of such conditions 
which give rise to abscess, or the absence of such conditions 
in the case of tumor. For other symptoms characteristic of 
abscess rather than of tumor, the student is referred to the 
next chapter. 

The ordinary forms of tubercular meningitis are so dis- 
tinct that they cannot be readily confounded with tumor of 
the brain, but the difficulty lies in the fact that some cases 
of tubercular affection of the brain take an exceedingly slow 
course, and that some forms of tumor prove rapidly fatal. 
Furthermore, tumor in the form of solitary tubercle and 
meningitis are occasionally associated with one another, as 
was proved in a case of mine of solitary tubercle of the 
corpora quadrigemina. In meningitis, whether of a tuber- 
cular character or not, the rapid involvement of a number 
of cranial nerves, without an increase in all the other symp- 
toms, will argue in favor of this disease rather than of 
tumor. This is true also of the specific meningitis as well 
as of the tubercular form.* 

Histological examination of the cerebro-spinal fluid, obtained by lumbar 
puncture, may assist in the diagnosis. Beware of lumbar puncture in cere- 
bellar disease ! The X-rays may occasionally be of service in locating a new 
growth (Mills, myself and others). 

Pathology. — The most important intra-cranial growth 
is the solitary tubercle. \i occurs both in children who ex- 
hibit distinct tubercular tendencies, and also in those who 
neither give a family history of tuberculosis nor exhibit 
any cachectic symptoms. 

In size the solitary tubercle may vary from that of a pea or cherry to one 
as large as that depicted in Fig. 122. In this brain it will be seen that a 
solitary tubercle had occupied the greater part of one hemisphere, and had 
considerably distorted the entire brain axis. Very often the tumor starts in 
the vicinity of the meninges, or at least near the cortical blood-vessels. The 
blood-vessels unquestionably play an important part, for in cases of tuber- 

* Oppenheim has reported several interesting cases of children presenting all the 
symptoms of a tumor of the Rolandic area, and ending in recovery. He thinks these 
may have been cases of meningo-encephalitis tuberculosa. Nonne has also described 
cases in adults and in youthful individuals supposed to be suffering from tumors of the 
brain ; all symptoms had receded. Hydrocephalus, serous meningitis, and specific 
lesions may cause difficulties in diagnosis. 



482 



THE NERVOUS DISEASES OF CHILDREN. 



cular meningitis of the base smaller tubercles of a pin-head size may be seen 
scattered along the vessels on the outer surface of the brain. A solitary 
tubercle is composed of a thick stroma with giant cells and a considerable 
amount of hard fibrous tissue. There is a more or less concentric arrange- 
ment of the parts, and the central portion of the tumor, in many cases, un- 
dergoes caseous degeneration at an early day. As in the cord, so in the brain, 
the tissue surrounding a tubercular growth may break down and become an 
almost diffluent mass. A tubercular encephalitis, or meningo-encephalitis, 
may, therefore, be superadded upon a solitary tubercle. In just as many 
cases, however, the solitary tubercle behaves as every other cerebral tumor 
does. It is, as a rule, sharply differentiated from the surrounding tissue, and 




Fig. 122. — Vertical Section through Cyst, C, and Both Tumors, showing Distortion of 
Brain Axis and Displacement of Left Ventricle. P, pons ; V, the left ventricle. 



the exact character of it can be made out only on histological examination. 
The very size of the tumor, as in the brain depicted in Fig. 122, is suspi- 
cious of solitary tubercle, for the smallest as well as the largest tumors occur- 
ring in the brain are of this character. In the histological examination of the 
tumor search should be made for the tubercle bacilli, and their presence will 
at once prove or disprove the supposed character of the tumor. 

Glioma and sarcoma, or a combination of both, are the 
next most frequent forms of neoplasm occurring in the brains 
of children. The glioma possesses the characteristics of neu- 
roglia tissue. Its growth is extremely slow, and for this 
reason it may be suspected in cases in which all the symp- 
toms point to a period of growth lasting for one or two 



TUMORS OF THE BRAIN AND ITS MENINGES. 483 

years, or even a longer period of time. In connection with 
glioma, as in other forms of tumor, there is, as a rule, a con- 
siderable increase in the blood-supply to surrounding parts. 
An increase of cerebro-spinal fluid is a constant accompani- 
ment, and it must be due to variations in the amount of 
these fluids that improvement sometimes follows upon treat- 
ment by iodides and mercurials. 

A patient of mine had been suffering for a long time from symptoms point- 
ing to a tumor in the parietal region ; he had also had an apoplectic attack some 
years previously. Taking these facts into consideration I concluded that the 
tumor was probably of a specific character, and placed him upon a very rigid 
iodide and mercurial treatment. The improvement was most marked, the 
headaches ceased, the convulsive seizures disappeared altogether, and 
the power in the right hand returned. I was convinced of the specific 
character of the tumor. The patient died, however, in a relapse, accompanied 
by an apoplectic seizure, and to my great surprise, on post-mortem examina- 
tion, the tumor, which was examined by Dr. Van Gieson, proved to be a 
glioma and not a gumma. It is of the utmost importance, therefore, not to 
attach too great significance to any improvement occurring in these diseases 
after specific treatment. 

Gliomata occur more frequently in the white than in the gray matter of the 
brain, but may invade the latter though starting from the white substance. 
The pons shares with the hemispheres the distinction of being the most fre- 
quent seat of glioma, the dense character of the tissue in the pons evidently 
yielding favorable conditions for the development of this special form of 
neoplasm. 

Sarcoma, pure and simple, or in connection with glioma, is somewhat less 
frequent than glioma alone. Its growth is more rapid than that of simple 
glioma, and it is not as distinctly separated from the healthy tissue as glioma 
generally is ; the tumor and infiltration as a rule extend for some distance into 
the neighboring tissue. By this invasion and by actual compression much 
of the tissue in the vicinity of a sarcoma is readily broken down. Round 
cells and spindle-shaped cells occur together with the fibrous mass, and 
according to the predominating character of the cells the sarcomata are di- 
vided into round-celled and spindle-celled varieties. Myxo-sarcoma is not 
unknown in childhood, and a few cases have been reported of that much 
rarer form known as melano-sarcoma. The sarcomata are invariably secon- 
dary to similar diseases in other organs. Sarcoma may therefore be sus- 
pected with malignant disease in other parts of the body, and particularly if 
the tumor be one of rapid growth, and if the symptoms point to an intensely 
destructive process. 

Cysts are extremely frequent in children, and are often found in brains 
not suspected of any gross disease. Such cysts are generally the remnants 
of an acute process early in life, an early hemorhage, or a softening due to 



484 THE NERVOUS DISEASES OE CHILDREN. 

embolism or thrombosis. These cysts fill up with fluid, and cause in the 
majority of cases no symptoms such as we assign to tumor ; but if from any 
cause such an old cyst is lighted up and its contents suddenly increase, the 
symptoms may resemble those of cerebral neoplasm. It is well to be pre- 
pared for the occurrence of such cysts, and to know that they are a favorite 
starting-point for other tumors. It was on the strength of such familiarity 
with the cerebral conditions of childhood that I was able to make the diag- 
nosis of a tumor growing from the cyst-walls in the case referred to on page 
569. 

Cystic tumors, in the true sense of the word, are those which are due to 
an invasion of echinococcus. In this country it is extremely rare to find a 
tumor of this description, but in Europe and in Eastern countries they 
are far more frequent. The cysticercus cysts occur in children as well as in 
adults, are multiple, and may give rise to a confusion of symptoms. 

The only other form of neoplasm which deserves special 
consideration is that due to syphilitic disease Gummata 
are unquestionably rare in children, but they may of course 
occur in cases of hereditary syphilis. We should look for 
them in connection with specific disease of the brain and 
its coverings. Repeated attacks and relapses would sug- 
gest the syphilitic character of the disease. 

Aneurism is also so rare in childhood that it would hardly need to be 
considered. But Osier reported some years ago an interesting case of aneu- 
rism in a child six years of age. The symptoms are very much like those 
caused by ordinary neoplasm, but the course of the disease is much 
more chronic than in the case of other new-growths. The diagnosis of 
aneurism could be reached only by exclusion, by the slow course of the dis- 
ease, and possibly by irregularities of the pulsations of the two carotid arte- 
ries. The anterior and posterior cerebral arteries are the vessels favored by 
aneurisms of any considerable size. 

In concluding these remarks upon the pathology of 
tumors, it is well to remind the student once more that the 
symptoms produced by any tumor are in part due to the 
tumor itself, to the character of the invasion, and to the 
region of the brain affected, and in part to the pressure 
upon the neighboring tissues, and upon distant parts of the 
brain structure. The increase of intra-cranial fluid plays a 
most important part in the causation of some of the symp- 
toms, notably of headaches and of optic neuritis. 

Treatment. — In spite of many advances in recent years, 
the medical or surgical treatment of brain tumors has been 



TUMORS OF THE BRAIN AND ITS MENINGES. 485 

disappointing. According to Starr's statistics only 7.5 per 
cent, of the cases are operable. Ferrier and Oppenheim 
give a similar percentage. Bramwell thought many years 
ago five out of twenty-two cerebral tumors could have been 
removed. In children the prognosis is still gloomier. For 
this there is a double reason ; first, tubercle is very much 
more frequent in children than in adults; second, if the con- 
ditions for the removal of the tumor are favorable, the child 
stands a poorer chance of surviving the operation than the 
adult does. Before proceeding to the surgical treatment of 
the case of brain tumor, which is, after all, the only thor- 
oughly rational course, the attempt should be made to in- 
fluence the growth, and to promote the absorption of the 
new-growths by various drugs. The most powerful drugs 
are the iodides and the mercurials. Gummata are very 
rare in children, and this treatment would therefore be of 
little use if it were given for its anti-syphilitic effect. But 
in every case of tumor there is considerable exudation, con- 
siderable increase of cerebro-spinal fluid, and both the 
iodides and the mercurials seem to have some effect upon 
the absorption of these products of inflammatory reaction 
in connection with tumor. By promoting such absorption 
of adventitious products the intense localized headaches 
and persistent vomiting, and other general cerebral symp- 
toms frequently present, are modified. Another reason for 
the employment of specific remedies is the belief that if they 
will not avail any in the actual cure of the trouble, they will 
at least help to relieve some of the most distressing symp- 
toms that occur in connection with cerebral neoplasm.* 

If any decided effect is to be hoped for, the iodides, as well as the mercu- 
rials, should be given in effective doses ; the iodides in doses varying from 
five to twenty-five grains three times a day ; the mercury, in the form of 
inunctions, from fifteen to thirty and forty-five grains twice a day, according 
to the age of the child. All other medicinal treatment will be of no avail. 

The headaches will be relieved best by a combination of phenacetine with 
codeine (one-fourth to one-third of a grain), or with small doses of morphia 
(one-sixteenth to one-eighth of a grain), according to the age of the child. 
Morphia alone I am not in favor of giving ; and if the pains are so extreme 

* Lumbar puncture might be tried in inoperable cases for the relief of pressure. 
Trephining for the relief of pressure is justifiable and beneficial. 



486 THE NERVOUS DISEASES OF CHILDREN. 

that the child becomes restless and sleepless in consequence of them, I should 
prefer moderate doses of chloral hydrate, from five to ten grains, according 
to the age of the child, given either per mouth or per rectum. If the tumor 
causes frequent convulsions the bromides might be added to the chloral, but 
it is best to avoid them in all cases in which epileptic seizures do not occur, 
for the bromides have no effect upon the intense headaches, unless given in 
very large doses, and have a disagreeable effect, interfering with the diges- 
tion and nutrition of the child. The ordinary hypnotics, such as sulfonal, 
trional, veronal, and the like, can be employed in moderate, doses in those 
cases in which there is a general restlessness without pain. 

In the first edition of this book the author took a dis- 
tinctly pessimistic view of the surgical treatment of brain 
tumors. Within the last few years the brilliant work of 
Horsley, of Kocher, of Keen, and the recent enthusiastic 
and ingenious writings of Frazier and of Cushing have 
given promise of far greater successes in this special field. 
Even the cerebellum has been made accessible. Sanger and 
Cushing are right in urging the great value of a palliative 
craniectomy as a means of " decompression " for the relief 
of headaches, and for the preservation of the eyesight. 
Within the last year this palliative operation has been per- 
formed for me by Gerster or Lilienthal in four cases of cere- 
bellar tumor occurring in children. The difficulties of sur- 
gical interference in children are, in part, similar to those 
met with in the adult, and in part peculiar to children only. 
Among the first we may mention the uncertainty which still 
prevails in every case regarding the exact size of a tumor, 
particularly if the tumor begins in one of the active areas of 
the brain and spreads to a so-called silent area. On this ac- 
count alone, however, no one would be justified in refrain- 
ing from an operation ; this uncertainty constitutes one of 
the attending risks. 

Among the conditions peculiar to children I would mention first of all the 
fact that children, according to my experience, which has been quite exten- 
sive, tolerate operations upon the brain very poorly indeed. The cerebral 
shock, and, above all, the considerable cerebral hemorrhage, lessen the chances 
of recovery very much.* If we consider, moreover, that a very large number 
of the cerebral tumors in children are of a tubercular nature, that multiple 

* The student is referred to the interesting articles of Frazier (N. Y. Med. Journal, 
February n and 18, 1905) and of Harvey Cushing (The Johns Hopkins Hospital 
Bulletin for March, 1905). 



TUMORS OF THE BRAIN AND ITS MENINGES. 487 

tumors are not infrequent, that the tumors occur with great frequency in 
the cerebellum and on the ventral surface of the brain, we are forced to the 
conclusion that there are but few cases of intra-cranial tumor in children 
which seem favorable for operation, and that of these few it must be a rare 
fortune indeed to have a single one recover completely. It. is the duty of 
every careful physician to weigh the evidence for and against an operation in 
every case, and if in a child of good physical strength a non-tubercular tumor 
is suspected in a region accessible to the surgeon's knife, the attempt at re- 
moval of such a tumor is justifiable if all other remedial measures have been 
given a previous trial. Whether a tubercular tumor should be removed or 
not is a question that cannot be decided off-hand. The answer may possibly 
be given some day by the results of operation in a case in which the character 
of the tumor was not suspected until it had been successfully or unsuccessfully 
removed.* 

* v. Beck has reported a case of tubercle of the brain successfully removed by 
Czerny. The author reports improvement after the operation. Some years ago Dana 
exhibited a boy of about 12 years old who had done well for more than a year after the 
partial removal of a sarcoma. 



CHAPTER XXVII. 
ABSCESS OF THE BRAIN. 

An abscess may be situated either on the surface of the 
hemispheres, or within the substance of the brain. The 
latter location is by far the more frequent. Multiple absces- 
ses sometimes occur. Bergmann found one hundred or more 
abscesses in a single case following gangrene of the leg. In 
one case the author observed at least six abscesses, which 
were successfully incised ; but a single abscess is more com- 
mon. If the pus is on the surface of the brain the mem- 
branes, which are generally thickened as the result of the in- 
flammatory process, may constitute one wall of the abscess. 

Abscess of the brain may occur at any period of life. Of the cases which 
Gowers has tabulated fully one-third occurred before the age of nineteen. 
The male sex is more disposed to this disease than females, in a ratio varying 
from two to one to five to one, according to the initial cause. The most 
frequent cause of abscess of the brain is preceding ear disease. Next in fre- 
quency is external injury to the skull. Gull and Sutton, in their article on 
abscess of the brain, in Reynolds's " System of Medicine," state that 102 
cases of abscess were due to ear disease, and 57 to injury. Koerner, in his 
exhaustive monograph on the otitic diseases of the brain, quotes Pitt's statis- 
tics, who records 56 cases of brain abscess among 9,000 autopsies. Of these 
56 abscesses 18 (very nearly one-third) were due to disease of the ear and of 
the petrous bone, while 8 (one-seventh of the entire number) were due to 
disease of other cranial bones. The relative number of other cerebral con- 
ditions complicating ear disease, as given by Koerner, is as follows : Phle- 
bitis and thrombosis of the sinuses occurred in 44 cases among 9,000 au- 
topsies, and of these 44 fully one-half were due to disease of the bony parts 
of the ear. A simple otitic meningitis as the result of purulent ear disease 
is relatively rare. Taking all cases of cerebral disease complicating otitis 
into consideration Koerner states that of 246 cases which he has been able 
to collect, 44 occurred before the age of ten, and 73 between the ages of 
eleven and twenty years. Thus it will be seen that fully one-third of all 
abscesses occurred during the period of childhood and early youth. Koerner 



ABSCESS OF THE BRAIN. 489 

states, basing his assertions upon Prussian statistics, that 5.15 per cent, of 
all deaths between the ages of ten and twenty were due to cerebral disease 
complicating otitis. These statistics will suffice to indicate the great inter- 
est that must attach to suppurative disease in early life, and particularly to 
abscess of the brain following upon disease of the ear. Other probable 
causes of abscess must not be neglected. Disease of the nose, empyema of 
the chest, and accidental injury to the skull, whether or not it has given rise 
to a tangible bone lesion, may be the starting-point of brain abscess. 

Gowers states that fifteen per cent, of all the cases of abscess of the brain 
were due to what he calls distant influences. Among these any condition 
giving rise to a general infection may be included, and it is more than prob- 
able that even among this fifteen per cent, of cases some may have been 
traumatic, although the exact character of the traumatism could not be de- 
termined. 

In abscess of the brain following upon chronic otitic 
disease the ear trouble may have existed for several years. I 
have known such ear disease to continue for ten years before 
the symptoms of abscess were developed. As long as there 
is a free discharge of pus outward, the danger of cerebral 
disease is comparatively slight ; but if the discharge is 
checked, the pus may burrow its way through the thin 
plate of bone separating the middle ear and the mastoid 
cells from the cranial cavity. The small veins which pass 
through the ear structures into the cerebral sinuses (supe- 
rior petrosal and the lateral sinus) provide easy means of 
access for the pus from the inner ear into the cranial cavity. 
From the mastoid cells the pus passes most easily into the 
lateral sinus. In connection with ear disease the abscess 
generally forms either in the temporo-spbenoidal lobes or 
in the hemispheres of the cerebellum. Usually it affects 
these regions, on the same side of the brain. In rare in- 
stances the chief formation of pus is on the side opposite to 
the diseased ear. 

The abscess formed in connection with ear disease is 
more often underneath the gray matter of the cortex than 
upon the outer surface of the hemispheres, and not infre- 
quently normal tissue separates the abscess from the carious 
bone and the meninges. Such being the case, it is evident 
that the abscess must have been formed within the brain 
and gradually extended outward. In a few instances a di- 
rect path of connection between the suppuration in the car 



490 THE NERVOUS DISEASES OF CHILDREN. 

and the abscess cavity has been demonstrated, but in the 
vast majority of cases no such connection has existed. The 
problem, therefore, is to decide in what manner the pus- 
corpuscles could have been carried from the ear to the in- 
terior of the brain. No entirely satisfactory solution has 
as yet been given, but the frequent interference with the 
circulation in the sinuses undoubtedly plays an important 
part. If we take into account the relative position of the 
temporo-sphenoidal lobes and the structures of the ear it 
will be seen that the superior petrosal sinus receives blood 
from both these parts, and, on the other hand, the lateral 
sinus receives blood from the cerebellum and the mastoid 
cells. It is due to these venous connections that abscess of 
the cerebellum is met with more frequently in connection 
with mastoid disease, and abscess in the temporo-sphenoidal 
lobes more frequently with disease of the middle ear. Such 
abscesses have been reported by Lucae, Koerner, Bruns, Mac- 
ewen, Gruening, Bacon, Dench, and many aural surgeons. 

Abscess of the brain in connection with diseases of the nose must be 
explained in much the same way (Dreyfuss). The nasal disease is sometimes 
limited to the mucous membrane without any involvement of the bone, yet 
abscesses form just the same. Under such conditions the toxic substance 
must be carried along the blood-vessels or the lymphatics. If the nasal 
bones are the seat of suppuration, as in syphilis, the abscess that forms may 
be in direct connection with the nasal cavity, and the pus from the brain may 
be freely discharged through the nose. 

The breaking down of tubercular growths may occasionally be the cause 
of abscess, but these cases have less practical interest, for the general tuber- 
cular character of the disease is the significant feature, and the abscess, as a 
rule, a mere incident in the course of a protracted illness. 

Purulent disease in any other part of the body may also be the cause of 
abscess of the brain through the agency of a septic embolus. Gowers quotes 
Boettcher's report of a case in which a cerebral abscess secondary to suppura- 
tion in the lung was found to contain lung pigment. There is a remarkable 
connection between brain abscess and every form of purulent disease within 
the thoracic cavity. Thus we have cases of brain abscess after pneumonia 
in which the exudate has not been perfectly resolved and has undergone sup- 
puration. Pus cavities occur after purulent bronchitis and empyema; re- 
cently Moschowitz operated upon a young boy in whom the author had 
helped to locate an abscess in the Rolandic area following empyema. 

As a rare cause of abscess, but one that is worth mentioning, we may note 
its occurrence after thrush in two cases reported by Zenker. One patient was 
an infant. In these cases the brain was studded with small abscesses con- 



ABSCESS OF THE BRAIN. 49 1 

taining the fungus of thrush (oi'dium albicans) ; the fungus had evidently 
travelled by way of the blood-vessels, since it had actually been demonstrated 
in their interior. 

Symptomatology. — Few conditions are more difficult to 
recognize during life than abscesses of the brain. It is safe 
to say that they constitute a large proportion of the sur- 
prises and disappointments of the post-mortem table. They 
are found when least expected, and when confidently looked 
for are often wanting. The uncertainty in the diagnosis is 
due to the very insidious development of the process and 
to the fact that when once encapsulated a pus cavity gives 
rise to very few symptoms. 

When fully developed an abscess practically constitutes 
a foreign body in the brain ; we would therefore expect 
such symptoms as are commonly due to tumors within the 
brain or cranial cavity, but in this we are apt to be disap- 
pointed, for the brain appears to accommodate itself to the 
slowly increasing abscess and does not respond to this 
growth as distinctly as it does to the invasion of a solid 
neoplasm. The abscesses do not, moreover, occur as fre- 
quently as tumors do in those parts of the brain which give 
rise to signal symptoms (the motor area and the occipital 
lobes). Abscesses are more common in the frontal lobes, in 
the temporo-sphenoidal lobes, and in the cerebellum. In 
acute cases, favorable for diagnosis, the first symptoms are 
those with which we are familiar in connection with menin- 
geal disturbances, such as local painfulness of the scalp, 
nausea, vomiting, vertigo, and fever. The last, if irregu- 
lar and if attended by rigors, is the one symptom which 
suggests abscess much more forcibly than any other. If 
the cavity is in the vicinity of the motor areas and is not 
too rapidly destructive, localized convulsions may occur. 
Abscess in this region, which is supplied by the middle 
cerebral artery, is very likely to be due to septic embolism ; 
hence the formation of pus in this region after lung disease 
and after septic endocarditis. Paralysis may be present in 
some of these cases if the motor area is involved, but if the 
abscess is elsewhere in the brain paralytic symptoms will 
not constitute a prominent feature of the disease until the 
terminal stage has been reached. 



492 THE NERVOUS DISEASES OF CHILDREN. 

To this category of symptoms, delirium, convulsions, ir- 
regular or high temperatures may be added, which lead to 
a rapidly fatal issue. 

Chronic abscess may exist for a number of years and 
may give rise to very few symptoms ; in one case of chronic 
abscess, in a boy of eight years, no symptoms were present 
as long as the ear freely discharged. As soon as this 
ceased, epileptiform convulsions, preceded by sensory auras 
(disagreeable odor and a hissing noise) would occur. This 
alternation was kept up for at least two years, when the 
boy died of a general purulent meningitis from bursting of 
the abscess into the temporo-sphenoidal lobe. The so- 
called latent state of the abscess is characterized, as a rule, 
by occasional spells of nausea, by occasional vomiting, and 
by intermittent febrile attacks for which no proper expla- 
nation can be found at the time. Persistent headaches, if 
associated with ear disease or with other conditions that 
give rise to abscess, point to the existence of this condition. 
The headache is generally more or less circumscribed, and 
sometimes varies according to the position of the head ; but 
this mere fact of change of position as affecting the head- 
ache does not necessarily point, as one would suppose, to 
abscess, for I have known the same condition to accompany 
solid tumor of the brain. The pain, as a rule, is referred 
to the vicinity of the abscess, but not invariably so. Mac- 
ewen's symptom (as described in Chapter XXVI.) may be 
of service, but should be utilized with great caution. The 
temperature is often lowered in abscess of the brain, and is 
accompanied by slow pulse. (Macewen.) 

Optic neuritis, so characteristic of tumor, also occurs in 
abscess of the brain. It is not as constant a symptom in the 
latter as in the former condition ; according to Okada's sta- 
tistics (quoted by Oppenheim) optic neuritis occurred in 
two-thirds of the cases. The neuritis is in nowise to be dis- 
tinguished from the neuritis associated with ordinary tu- 
mors within the cranial cavity. The other symptoms will 
vary very much according to the location of the abscess. 
Paralysis, if present, is apt to be hemiplegia If associated 
with unilateral convulsions it points to the motor cortex as 
the site of the abscess. Sensation is, as a rule, not involved, 



ABSCESS OF THE BRAIN. 493 

and the various cranial nerves are not affected unless the 
abscess is at or near the base. The fifth and sixth nerves 
may be pressed upon by a cerebellar abscess, causing- pain 
in the face and paralysis of the muscles of mastication 
and of the rectus externus. Cerebral abscess may also be 
associated with paralysis of the facial nerve, but this is only 
rarely due to involvement of the nerve as it emerges from 
the pons, but is more frequently of old standing and due to 
an early disease of the ear affecting the nerve in its course 
through the Fallopian canal. Paraphasia, amnesic aphasia, 
alexia, agraphia, accompany abscess involving parts con- 
nected with the function of speech. In several patients 
whom I had the privilege of seeing with Gruening, sensory 
aphasia was the first signal symptom of an abscess in the 
temporo-sphenoidal lobe. Hemianopsia may be present 
(case of Knapp). Stupor, delirium, and coma mark the ter- 
minal stages of abscess. In cerebellar abscess the charac- 
teristic signs are: Pain over the occiput and the region of 
the neck; rigidity of the neck; vertigo, inco-ordination and 
typical cerebellar ataxia ; vertigo and vomiting when the 
position of the head is changed (Bruns); bulbar symptoms. 
Differential Diagnosis. — From the account of the 
symptoms accompanying abscess of the brain it is evident 
that it is difficult, sometimes impossible, to differentiate 
between a pus cavity in the brain and other conditions. 
Among these are solid tumor of the brain, meningitis, and 
meningo-encephalitis. The diagnosis of solid tumor can 
more readily be made if the usual causes for abscess, such 
as ear disease and external injury, are wanting, and if the 
symptoms have developed in a subacute fashion, which 
is rather characteristic of tumor, while in abscess there is 
either a prolonged latent period or all the symptoms de- 
velop in a very short period of time. Suspicion of abscess 
is strengthened above all things by the presence of marked 
rigors and rapidly changing temperatures. A slight change 
of temperature may occur in the case of tumors, but it is 
not as variable and does not show the extremes so common 
in abscess. During the past years I have observed several 
cases of abscess in which there was not the slightest rise of 
temperature for several weeks preceding death. 



494 THE NERVOUS DISEASES OF CHILDREN. 

The differential diagnosis between tumor and abscess is one of practical 
importance. If the new-growth is near the surface, and if the symptoms are 
of such a kind as to lead one to infer a tumor of considerable extent, the 
surgeon might well hesitate to operate if he thought the new-growth a solid 
one. If there is a probability of an abscess the size of the abscess need not 
constitute a contraindication to operation, and this differentiation between 
tumor and abscess is still more important if the growth is supposed to be in 
the interior of the brain. If this should be a solid tumor a surgical opera- 
tion would do no good, but if it is an abscess it could well be drained through 
the hemispheres, and the greatest danger of all, rupture into the ventricles, 
might be averted. 

The differentiation between meningitis and abscess is not an easy one, 
particularly in cases of ear disease and external injuries. Meningitis is also 
apt to be associated with, or to precede, the formation of abscess, and during 
this period the question whether the suppurative, and hence the operative, 
stage has been reached is not always easy to decide. Moreover, in the final 
stages of abscess, particularly if the abscess has ruptured, a general purulent 
meningitis is the invariable result. At that stage a differential diagnosis can 
easily be made with a great degree of certainty, but if made has little practical 
value. In the earlier stages of cerebral disease after otitis or external injuries 
it is safe to believe that the condition is still one of meningitis, without forma- 
tion of abscess, if the symptoms are those of a general cerebral disturbance, 
such as vertigo, headaches, nausea, slight stupor, but without rigors or serious 
rise of temperature. Localizing symptoms may be present in both cases. 

The distinction between meningitis and abscess is of much practical im- 
portance for another reason. The majority of surgeons would not be willing 
to operate upon cases of meningitis ; they evidently have the general puru- 
lent meningitis in mind ; with improved methods in surgery, exploratory 
trephining may be warranted, particularly in connection with ear disease or 
external injury if there is a suspicion of meningitis alone, provided the latter 
be limited in extent. Lumbar puncture may give diagnostic aid. 

In practice the differential diagnosis most frequently to be established is 
between abscess and sinus thrombosis after middle-ear disease. The gen- 
eral cerebral symptoms may be similar in both conditions, but the special 
symptoms of sinus thrombosis (see p. 498) will help to decide the point. In 
many cases the condition is uncertain before the operation is undertaken. 
In every doubtful case the surgeon should look first for sinus thrombosis, 
and then for abscess. 

Prognosis. — The prognosis of abscess of the brain is in- 
variably grave. While admitting the possibility of an ab- 
cess becoming encapsulated and of remaining latent in the 
brain for many years, the probability is in every instance 
that serious mischief will result from it sooner or later. 
The physician must not trust to good fortune in such dis- 
eases, but knowing the serious nature of the trouble he is 



ABSCESS OF THE BRAIN. 495 

bound to present to the patient, or the patient's relatives, 
the alternatives of surgical interference or death within a 
limited period of time. The longer the abscess has lasted 
the more serious it is apt to be, for the danger of rupture 
becomes greater with the increase in its size. 

Treatment. — In spite of recent advances in cranial sur- 
gery and of the successes that have been recorded in the treat- 
ment of abscess of the brain it is best to discuss the proph- 
ylactic measures which may possibly prevent the formation 
of abscess. Knowing that pus is formed most frequently 
after ear disease and after disease of the bone, the greatest 
caution should be exercised to prevent any extension of the 
inflammatory process into the cranial cavity from these 
original sources of infection. The possibility of cerebral 
complications in every case of suppurative ear disease 
should be kept well in mind, and free discharge outward 
should be secured and maintained. In the case of mastoid 
and middle-ear disease we are not only bound to secure a 
free passage outward for the pus, but the discharge should 
be watched, and as soon as the pus ceases to flow outward 
while the symptoms point to a constant generation of pus 
in the bony structures, surgical measures should be resorted 
to to drain away the pus that is formed. While pus is dis- 
charging freely from the internal or the middle ear, if symp- 
toms arise pointing to meningeal or cerebral involvement, 
such as increasing headaches, vertigo, vomiting, and fever, 
further surgical aid is needed. 

An attempt may be made when the first meningeal 
symptoms arise to treat these in the ordinary way by the 
application of cold, by blistering, by counter- irritation, 
and the application of leeches and the like, but very little 
time should be wasted with such measures, and if the 
symptoms do not yield within twenty-four or forty-eight 
hours the surgeon must afford the needed relief. In the 
case of abscess due to ear disease, until recently little suc- 
cess has attended the efforts of the surgeon to locate and 
drain the abscess; but it bids fair to become the most satis- 
factory and legitimate cause for operation upon the brain. 
This opinion (of the first edition) has received fullest indorse- 
ment by the experience of recent years. 



49<5 



THE NERVOUS DISEASES OF CHILDREN. 



The temporal convolutions can be exposed by trephining 
at a point which is reached by drawing a Jine one and a 
quarter inch back from the external auditory meatus, and 
drawing another, at right angles from this one, the same 
distance upward. The terminating point of this line may 
be made the centre of the trephine opening, and will be 
sufficiently accurate to enable the surgeon, after removing 
a considerable portion of the bone, to expose the region 
of the abscess. As the abscess is not always superficial, 
punctures should be made with a fine hypodermic needle in 
various directions in order to reach cavities that are situated 

below the gray mat- 
ter. By observing 
these rules one can 
hardly fail to find the 
abscess. In one case 
of the author's, the 
surgeon had to make 
four punctures into 
the substance of the 
brain before pus was 
withdrawn. Absces- 
ses formed in connec- 
tion with disease of 
other parts of the cra- 
nium can generally be found if one is guided by the external 
signs ; thus, in a case which was operated on for me nearly 
ten years ago, there was caries in the middle of the parietal 
bone. The surgeon was directed to open the skull at this 
point, and, if no abscess presented, to insert the needle in 
various directions. When the dura was exposed nothing ab- 
normal was noticed, but the first thrust of the needle secured 
a large quantity of pus ; the brain tissue was then incised 
and the entire abscess cavity opened, but the patient died a 
few days later, and on the post-mortem table other abscesses 
were found in the opposite hemisphere of the brain as well 
as at the base. If multiple abscesses can be diagnosticated, 
operative interference is useless. In cases of skull injury 
the trephine opening should always be made at the seat of 
the external injury, and enlarged from this point to meet 




Fig. 123 — Dissections showing the Guide Adopted 
by Barker in Successful Trephining for Abscess 
from Ear Disease. (After Gowers.) 



ABSCESS OF THE BRAIN. 497 

the exigencies of a given case. If the pus cavity be due to 
disease of the nose, a large opening in the frontal bone 
should be made, from which the surgeon will then attempt 
to locate the exact seat of the abscess, and, if possible, drain 
it according to the usual method. 

Abscesses in the cerebellum are now within reach of 
the surgeon's knife. Koerner has collected 55 cases of 
cerebellar abscess, with a favorable result in 52.8 per cent, 
of the cases. It is well, however, to remember that the 
majority of unsuccessful cases are not recorded. 

Successful treatment of brain abscess has been reported by Stimson, 
Bergmann, Schede, Paget, Park, Koerte, Jansen, Knapp, Gruening, Starr, 
Saenger and a host of others. The case of Knapp is of special interest, as 
it occurred in a child nine years of age. The chief symptoms were chronic 
otorrhcea (after scarlet fever), optic neuritis, homonymous hemianopsia, and 
Macewen's symptom (percussion note stronger on left side) ; there was no 
sensitiveness on percussion of the skull. At the operation a pus cavity was 
found, and complete recovery ensued in the course of several weeks, after 
minor mishaps. 

There is no reason why children who present indubitable 
signs of brain abscess should not be operated upon according 
to the same principles which hold good in adults.* It is 
probable that with greater experience in these cases the 
operation need not last several hours, as in some of those 
hitherto reported, and hernia cerebri can surely be avoided. 

THROMBOSIS OF THE INTRA-CRANIAL SINUSES. 

The blood circulates in the venous sinuses within the skull under special 
disadvantages. The walls of the sinuses are rigid, the blood cannot be emp- 
tied during inspiration, and many of the cerebral veins join the longitudinal 
sinus at an obtuse or right angle, so that their blood is poured into the 
superior sinus in a direction opposite to the main current. Under such con- 
ditions it is natural that the current should be retarded and coagulation of 
the blood easily set in. 

Two forms of thrombosis occur ; the first is the primary or marasmic 
thrombosis which occurs in children after exhausting diseases (cholera infan- 
tum, etc.). This is generally confined to the longitudinal sinus, but may 
involve the lateral and cavernous sinuses. In such cases the clots are dense, 
resistant, organized, and do not adhere to the wall of the vein. The throm- 

* The readiness of the aural surgeons to open up the mastoid will do much to 
diminish the frequency of brain abscess. 



498 



THE NERVOUS DISEASES OF CHILDREN. 



bosis may be limited to the cerebral veins, thus giving rise to limited or cor- 
tical symptoms. Gowers, it will be remembered, has attached special signifi- 
cance to this condition in the causation of infantile cerebral palsies. 

The second form is the secondary or infective thrombosis, which generally 
affects the lateral, the cavernous, or the transverse sinuses. It is secondary 
to some infective process in the neighboring tissues or at a distance. Middle- 
ear disease is the most frequent cause, but it may also be due to some trau- 
matism of the skull (infected wounds), erysipelas of the head and face, to 
purulent disease of the eyes and of the nose. In one of the author's cases 
caries of the jaw was the starting-point of sinus thrombosis. In other 




Fig. 124. — Girl, Aged Twenty. Exophthalmus, with internal strabismus; oedema of 
the right eyelids, side of the nose, the brow, and the face. Later in the disease the 
left side was also affected. Thrombosis of cavernous sinus. (Macewen. ) 



cases the infectious material is carried along the veins and through neighbor- 
ing tissues. 

The inflammation may extend directly to the walls of the sinus and thus 
cause clotting of the blood within, or the clot may form within a vein and 
extend from there into the sinus. The superior petrosal and the lateral 
sinuses receive their blood from the middle ear, hence the frequency of throm- 
bosis of these sinuses in middle-ear disease. Thrombosis of the sinus result- 
ing from actual compression is very rare indeed and of little significance, as 
the symptoms would be obscured by those of the primary affection. 

Symptoms. — The symptoms of sinus thrombosis are often complicated 
by those of the primary disease. We must distinguish between general and 
special symptoms. Among the former manifestations the most important are 
intense headaches, somnolence increasing to stupor and coma, convulsions, 
slight rigidity of the neck, optic neuritis, rigors, accelerated or diminished 



ABSCESS OF THE BRAIN. . 499 

pulse-rate, thready pulse, and fluctuating temperatures ; but most of these 
symptoms would suggest meningitis or encephalitis quite as readily, if the 
conditions favoring sinus thrombosis were not known to be present ; more- 
over, actual meningitis may be caused by thrombosis of the sinus, particu- 
larly if such thrombosis is of an infective nature. 

The special symptoms refer particularly to disturbances in the venous 
circulation and to areas of tenderness. Thrombosis of the superior longi- 
tudinal sinus is characterized by cedema of the scalp and distention of the 
veins in the parietal, frontal, and occipital regions. In infants the anterior 
fontanelle becomes prominent, and epistaxis is frequent; squint is a rare 
occurrence. Cavernous sinus thrombosis is rare (the author has seen two 
cases within a single year) ; the thrombosis is generally due to extension 
from other sinuses, from the petrosal or lateral sinus, or it may result from 
disease of the ophthalmic veins (Knapp) and abscess in the orbital cavity. 
In more than half of the cases the thrombosis spreads to the cavernous sinus 
of the opposite side. The special symptoms are exophthalmus, cedema of 
the eyelids and root of the nose due to venous obstruction ; also ptosis, paral- 
ysis of rectus externus, and supraorbital pain, all due to compression of various 
nerves to the orbit running close to the sinus. (See Fig. 124a.) "Choked 
disk " from obstruction of the retinal and ophthalmic vessels is present in 
some cases of cavernous sinus thrombosis. Defect of vision and amaurosis 
may be superadded. Some of these cases live five or nine months, but all 
end fatally. The author had a case under observation in which the condition 
has remained practically unchanged for nearly two months. 

Thromboses of the petrosal and lateral sinuses are the most frequent, and 
the most important ; unfortunately they present very few special symptoms. 
In lateral sinus thrombosis there may be distention of veins and cedema over 
the mastoid process, and tenderness on percussion in this region, but the 
symptoms differ very little from those due to ear disease and localized men- 
ingitis, and all three conditions may be present. Inflammation of the veins 
leading from the lateral sinus occurs in cases of infective thrombosis. When 
these veins are inflamed pain may be elicited along the internal jugular vein, 
and over the upper third of the posterior cervical triangle. A difference in 
the sensitiveness of the two sides will be strong corroborative evidence of 
suspected thrombosis. Cervical abscess and enlargement of cervical glands 
may result from infective external thrombosis of the lateral sinus. 

The prognosis of sinus thrombosis is always grave. A fatal issue is to 
be expected, although death may be delayed for a period of several months. 
The only hope lies in successful surgical interference and in early preventive 
measures. A few recoveries are on record. The lateral and transverse 
sinuses have been opened successfully and the clots removed.* Ballance, 
Sulzer, Schwartze, Parkin, Horsley, Macewen, Bacon, Pritchard, and Cheadle 
have reported successful cases. Parkin's and Cheadle's patients were respect- 
ively nine and thirteen years of age. Henschen has collected 145 cases of 

* The details of the operation should be studied in Macewen's treatise, page 307 
et seq. 



500 



THE NERVOUS DISEASES OF CHILDREN. 



operation for sinus phlebitis following otitis ; of these, 85 were cured. Tying 
the jugular vein before removing the clot seems to exert a favorable influence 
upon the percentage of cures. Koerner states that there was recovery in 
seventy-five per cent, of the cases if the jugular vein was tied, and only fifty 
per cent, recovered if the vein was not tied. Jansen does not favor this 
procedure in all cases. 




.Torcular i 
Fig. 124a. — The Veins of the Dura Mater. (Heitzman. ) 



The extensive experience of aurists and a far larger collection of cases 
have helped to decide many mooted points, and the successful operations 
recorded encourage one to hope that sinus thrombosis (excepting possibly as 
part of a general pyaemia) will become an eminently curable disease. Explora- 
tory operations in doubtful cases are not only justifiable but imperatively 
demanded. 



CHAPTER XXVIII. 

DISEASES AND CONDITIONS DUE TO DEFECTIVE DEVEL- 
OPMENT OF THE BRAIN. 



LARGE DEFECTS. 



Just as there are abnormalities of the spinal cord, due to defective closure 
of the vertebral arches, so there are deformities of the brain and its coverings 
due to defective closure of the skull. The various forms of protrusion are 
best illustrated by Figs. 125-127, taken from Hob's Textbook. These de- 




Fig. 125. — Meningocele. 



Fig. 126. — Encephalocele. 
(After Holt.) 



Fig. 127. — Hydrencephalocele, 



formities are rare and of little interest, although Treves and others have 
attempted to remedy these conditions by surgical means. Most of the chil- 
dren thus afflicted die within a few weeks. 

In meningocele, the membranes only are protruded ; in encephalocele 
some brain substance is protruded ; in hydrencephalocele the protruding brain 
substance contains a cavity which is in direct communication with the dis- 
tended lateral ventricle. These tumors, varying much in size, may be occip- 
ital or frontal ; they may be covered by the scalp, or only by granulation tis- 
sue ; other deformities are often associated with them. 

Cyclops or monopus, as the name tells, indicates a condition in which 
but one orbit exists in the middle of the face, and about in the position gen- 
erally occupied by the root of the nose. The size of this one orbital cavity 
varies according to the development of the eye. In some cases the eye is 
not present, and the orbital cavity is correspondingly small. The cavity at- 
tains its largest size if the two eyes are fused into one ; and in accordance 
with the single or double development of the eye, one or two optic nerves may 
be present. If no bulb has been developed there is also a corresponding de- 
fect of the optic nerve. The eyelids may be single or double, the nose, as a 



502 THE NERVOUS DISEASES OF CHILDREN. 

rule, is undeveloped, and its place indicated by something which simulates 
a short snout. The entire features are distorted, the mouth may be wanting, 
and the ears come closely together. 

The only reason that we have to consider this condition at all is that it is 
due to a defective development of the anterior cerebral vesicle ; the ganglia, 
too, are more or less deficient ; the corpus callosum may be wanting alto- 
gether. The parts of the brain coming from the middle and hind brain vesi- 
cle are, as a rule, better developed than the hemispheres are. 

Fortunately these cases are not viable ; they have therefore no clinical 
value whatever, and may well be relegated to the authors specially interested 
in monstrosities. 

The next most frequent and most complete anomaly of brain structure 
is the condition known as anencephalus or hemicephalus. The difference 
existing between the two conditions consists, as the names indicate, in the 
complete or partial absence of brain development. Steffen assumes, without 
decided evidence, that these departures from normal development are due to 
an inflammation of the meninges occurring during the foetal period, in conse- 
quence of which a large amount of cerebro-spinal fluid collects in the ventri- 
cles, in the meshes of the pia, and in the subdural spaces ; and that in conse- 
quence of this increased pressure upon the developing parts the normal 
growth of the brain is interfered with. There is no reason to dispute this 
view until some better opinion is offered. The only suggestion to be made is 
that the inflammation may have been preceded by disturbances of circulation, 
and if such disturbance of circulation is the important factor, we shall have 
the same cause operative in these cases of larger defects as in those smaller 
defects called porencephaly, which we shall study hereafter. In cases of an- 
encephaly or hemicephaly a very considerable portion of the skull may be ab- 
sent. There may be rudiments of the parietal, of the temporal, or of the 
occipital bones. In some cases a portion of the occipital bone is left, arid 
this, together with the petrous portion of the temporal bone, provides a small 
groove in which some cerebellar rudiments may be lodged. The frontal bone 
is, as a rule, undeveloped. If present, it is very low, the eyes therefore pro- 
trude, the face points upward, and the jaws push forward. Instead of the 
roof of the skull, a thickened membrane may be all that is present. In other 
cases the bony defect may be covered by a tolerably normal scalp, which 
may be fused with the dura mater. 

If the skull is opened every conceivable defect may be found. In the ma- 
jority of cases after removal of the bone, the thickened membranes present 
themselves, and if these are punctured a very large amount of fluid flows out, 
the membranes collapse, and further examination of the specimen may show 
nothing excepting the basilar structures, no trace of the hemispheres or 
any part of the frontal brain being present. 

PORENCEPHALY. 

The condition of porencephaly is much more interesting from every point 
of view than the larger brain defects previously mentioned. The term por- 



DEFECTIVE DEVELOPMENT OF THE BRAIN. S°3 

encephaly has been much abused. It has been made to embrace an entire 
absence of brain structure, or very minute defects in the brain, secondary to 
vascular lesions. It would be best to use the term in the way it was first 
used by Heschl, who invented it, and by Kundrat, who wrote the first exten- 
sive monograph on the subject. As the name indicates, it is practically a 
hole in the brain, not an entire absence of brain structure. For that reason 
there should be some evidence of development of that part of the brain in 
which the hole lies. This hole, or porus, may be the result of defective de- 
velopment, or it may be acquired in the latest period of fcetal life, or even in 
the first period of life after birth. It would be well to distinguish the truly 
congenital cases from the acquired cases, with the additional qualification 
that even the truly congenital cases may be acquired in the sense of being 
the result of some morbid process during the fcetal period. 



Congenital Porencephaly. — The defect may be in the 
anterior, in the middle, the posterior, or, in fact, in any 
part of the brain, but it is found much more frequently in 
the anterior and in the middle portion. Congenital poren- 
cephaly may be single or double. 



An extreme condition of porencephaly will be best illustrated by Figs. 128 
and 129, taken from Schultze's monograph, which he entitles " A Contribution 
to the Doctrine of Congenital Cerebral Defects." I select this case because it 
represents, as it seems to me, an extreme condition of porencephaly, and is 
only one stage short of a defective development involving a very large portion 
of the entire brain. By reference to the figures it will be seen that the posterior 
half of the brain is tolerably well developed, but that the anterior half is 
practically nothing but a sac enclosed by a thick membrane. The defect in- 
cluded not only the two frontal and a considerable portion of both parietal 
lobes, but the greater part also of both temporal lobes. The posterior half of 
the brain, the cerebellum, and all the ganglia proved normal on microscopical 
examination. Schultze describes this brain very thoroughly, and it is of es- 
pecial interest inasmuch as its bearer lived to the age of five years. As far 
as any history may be depended upon, it was simply this, that the child 
appeared normal at birth, and was supposed to have moved its hands ; but 
this statement Schultze questions, and states that later on both arms 
were in a condition of flexion and adduction. The legs were flexed, and 
were in a state of spastic contracture and adduction. The child never had 
any convulsions, but was able to scream and to suckle. The father of the 
child noticed that there was no sign of intelligence, that the child would not 
take hold of anything, that it did not hear, and that it lay constantly with its 
eyes rolling about, and with its head retracted. The condition was, therefore, 
one of complete idiocy, with paralysis and contractures of both upper and 
lower extremities. The child grew weaker and weaker, and finally died of a 



504 



THE NERVOUS DISEASES OF CHILDREN. 



bronchitis. The parents of this child had six others, all of them healthy. 
The mother of the child was addicted to drinking. 

Schultze disputes the correctness of Kundrat's views, that porencephaly 
is the result of anaemic necrosis. This author insisted on the marked 
distinction between the congenital and acquired cases of porenceph- 
aly. If the porencephaly is of intra-uterine origin, the convolutions are so 
arranged as to radiate toward the margin, or toward the middle of the 
defect. If the defect is an irregular one, and the convolutions are not ar- 
rayed in any definite fashion the porencephaly is acquired after birth. In 



A. C. C. 




P. C. C. 



Fig. 128. — Brain with Large Anterior Defect. A. C. C. anterior. central convolutions; 
P. C. C. , posterior central convolutions. (Schultze.) 



this question, as in so many others, there is some truth in views that appear 
to he distinctly divergent. I have no doubt that some cases of porencephaly 
are due to vascular lesions, and others to inflammatory processes which may 
possibly start from the meninges. The symmetrical defect in Schultze's case 
seems to me more likely to be due to an inflammatory meningeal process 
without exudation, at a relatively early stage of fcetal development, than to a 
destruction secondary to vascular disease. In Schultze's case, moreover, the 
vascular apparatus was found to be entirely normal. The question arises, 
What the causes of an intra-uterine inflammatory process could possibly be ? 
If the mother had passed through any pyaemic condition, or had had some 
form of purulent disease during pregnancy the cause of such inflammatory 
meningitis or encephalitis would not be far to seek ; but in Schultze's, as in 



DEFECTIVE DEVELOP MEXT OF THE BRAIN. 



505 



other cases, no such cause could be made out, and it will not do to speculate 
upon improbabilities. The readiest explanation is to assume, as many of the 
older French writers assumed with regard to various brain defects, that 
traumatism plays a very important role, and that traumatic encephalitis dur- 
ing the intra-uterine period may be the actual cause of some of these poren- 
cephalic conditions. Schultze mentions that spasmodic contractions of the 
uterus without any extraneous disturbance may be a possible cause of such 
traumatic changes in the fcetal brain. In his own case Schultze is inclined to 
consider the possibility of a degeneration or obliteration of some of the 




Fig. 129. — Same Brain as in Preceding Figure, Viewed from the Base. (Schultze.) 



blood-vessels, or even of an encephalitis, as a direct result of alcoholic ten- 
dencies of the mother. In the most recently reported cases of porencephaly 
of the parietal region there seems to be atrophy of all motor fibres from the 
cortex to the medulla oblongata. (In eleven of fourteen cases. — Anton.) 

Acquired Porenxephaly is a condition due to early meningeal 
hemorrhage. This in turn is the result of compression of the skull during 
labor. The symptoms associated with these brain conditions will be found 
described under the heading of Cerebral Birth Palsies. The meningeal hem- 
orrhage is in many cases most severe under the parietal eminences, and over 
the motor areas. For this reason double spastic cerebral palsy is a natural 
result of a defect which develops in the region occupied by the motor cen- 
tres. This region is also supplied by the middle cerebral artery, which is 
most liable to all vascular disease. The porencephalic defect due to men- 
33 



506 



THE NERVOUS DISEASES OF CHILDREN. 



ingeal hemorrhage may be present in both hemispheres, or it may exist in 
only one-half of the brain. The meningeal hemorrhage, if sufficiently se- 
vere, leads to a compression of the cortical substance ; atrophy of the cortex 
follows, upon this the substance shrinks considerably, and the cerebro- 
spinal fluid is increased either as a result of inflammatory action, or of an 
effort at compensation. The pia and the cortex are apt to become aggluti- 
nated, and thus local cystic 
conditions with extreme loss 
of substance may readily oc- 
cur, the cyst occupying the 
place which should be filled 
by normal brain substance. 

Through the kindness of 
Dr. Peterson the author is 
enabled to insert an illustra- 
tion (Fig. 130) of the brain of 
a child that died at the age 
of twenty months. The child 
had been subject to convul- 
sions and had presented com- 
plete spastic paralysis of all 
four extremities ; the head 
measurements were extreme- 
ly small (circ. 32.5 ctm.). Over 
each hemisphere a large por- 
tion of the convolutions was 
wanting. The vacuum was 
filled partly by subdural fluid, 
and partly by the bulging of 
each ventricle, for there was also internal hydrocephalus. Peterson thinks the 
condition due to a double meningeal hemorrhage, but the defect corresponds 
so nearly to the distribution of the middle cerebral arteries that obliteration 
of these blood-vessels early in foetal life may have been the prime cause. 




Fig. 130. — Large Double Porencephalic Defect. 
Child lived to age of twenty months. (From a 
brain kindly furnished by Dr. Peterson.) 



MICROCEPHALUS. 



In addition to the anomalies of brain structure in which 
one or more parts may be defective, we have to consider 
that condition in which the brain as a whole shows a de- 
fective development, failing to attain to the normal size. 
There is, as a rule, a correspondingly small development of 
the skull, and to this small head the term " microcephalus " 
is given. While a microcephalus is bound to harbor an ab- 
normally small brain, we must not forget that a skull of 



DEFECTIVE DEVELOPMENT OF THE BRAIN. 507 

normal size may also contain a small brain, the cranial 
cavity under such conditions being filled up by an excess 
of fluid. Microcephalus is evidently the result of a num- 
ber of different processes. It has been a habit to explain it 
altogether on Virchow's theory, that the smallness of the 
skull was due to a premature ossification of the cranial 
sutures, which interfered with the proper expansion of the 
skull, and that in consequence of this stunted growth of 
the skull the growth of the brain was arrested. This ex- 
planation will hold good for a considerable number of cases, 
but I am firmly convinced not tor all. We must allow also 
for those cases of microcephalus due to defective cerebral de- 
velopment during the intra-uterine or during the early extra- 
uterine period. For reasons which we cannot always fathom, 
the brain does not grow and the skull remains small. The 
children are small-skulled and small-brained. In another 
series of cases the microcephalus is, I am convinced, the 
result of early vascular lesions. I have in mind several 
cases of children who were born normally, whose cerebral 
capacity was on a par with that of other children up to 
the age of six, seven, or nine months. Then convulsions 
set in, probably resulting in extensive meningeal hemor- 
rhage, or, let us say, in an extensive pachymeningitis hem- 
orrhagica. In consequence of this the cortex was com- 
pressed ; it became atrophic and sclerotic and, remaining 
small, the skull did not expand in normal fashion. Such 
children become microcephalic after the first year of life, 
or later. 

This leads us to a question which has been much discussed and unsatis- 
factorily answered, whether the growth of the skull depends upon the growth 
of the brain or vice versa (Meynert, Virchow, Lucas). The two hold a 
very close relation to one another. If premature stenosis sets in it is un- 
questionably sufficient to account for a small brain ; but, on the other hand, 
a growing brain evidently helps to expand a normal skull. The fact that 
defective brains are often covered by normal skulls if the defect has been 
compensated by a local accumulation of fluid, would seem to prove that as 
long as the same mechanical conditions are present the growth of the skull 
remains tolerably normal (Gudden). 

In order that we may be able to determine when a skull 
may be termed microcephalic, we must refer to the meas- 



508 THE NERVOUS DISEASES OF CHILDREN. 

urements of normal heads at certain ages (see Introductory 
Chapter). If a head which is supposed to be microcephalic 
fall far short of such average measurements the diagnosis 
of microcephalus may be safely made ; but the head may 
be developed in proportion to the rest of the body ; a small 
measurement in a child need not necessarily imply a con- 
dition of microcephalus, unless the measurement of the 
skull is below, while other measurements of the body are 
fully up to, the average in other children. 

The general measurements of the skull are not always sufficient to prove 
whether the brain is small or not. Each part of the skull should be inspected 
per se. Thus I have seen extremely small and receding foreheads with un- 
usually large occiputs. This would tend to give a measurement of a general 
circumference quite up to the standard, yet on closer inspection there would 
be no doubt that the frontal portions of the skull and of the brain were poorly 
developed, whereas the occipital portions were either normally developed or 
caused to bulge by the accumulation of hydrocephalic fluid within the ven- 
tricles. 

In several other heads which the author has examined this defect in the 
anterior portion was extremely marked, and as the frontal portion of the 
brain has least to do with motor and sensory functions, and more with the 
general intellectual development of the child, we can see the important bear- 
ing such facts have upon the prognosis of a child's future mental develop- 
ment. A frontal microcephalus may be present even though the cranial 
measurements be up to the average. 

The symptoms due to microcephalus are described 
under the heading of idiocy, and in the chapter on the Cere- 
bral Palsies of Children.* In this connection we may refer, 
however, to efforts made within recent years to correct 
these cranial deformities on the supposition that if the size of 
the cavity could be enlarged the brain would have a more 
favorable opportunity for further growth. Such reasoning 
could apply merely to those skulls in which the chief 
trouble lies in the premature synostosis of the sutures. If 
there is an inherent defect in the development of the brain, 
or if the brain growth has been checked as the result of ac- 
quired disease, such treatment is not applicable. The great 
difficulty, however, is, that it is not always easy to deter- 

* Of course microcephalic heads are at times found in persons of tolerable cerebral 
development. 



DEFECTIVE DEVELOPMENT OF THE BRAIN. 509 

mine to which one of these causes the defective cerebral 
development is due. Lannelongue suggested many years 
ago that in the case of microcephalic skulls a longitudinal 
opening be made in one or both halves of the skull, thus 
practically increasing the size of the intra-cranial cavity, or 
at least diminishing the pressure within this cavity. My 
experience has not been at all favorable to Lannelongue's 
doctrine, and I am willing to say that there is not the least 
reason to advise or encourage the operation, at least accord- 
ing to the method proposed by Lannelongue. This French 
surgeon operated upon a number of skulls, and claimed that 
after operation the children showed some signs of increased 
intelligence and were greatly benefited by the operation. 
Other surgeons have had but few favorable results to show, 
and of the cases operated upon there were not many in 
which a small brain was due to smallness of skull. I have 
had three children operated upon, and on one patient two 
operations were performed. Two patients with micro- 
cephalic skulls due to premature ossification of the sutures, 
died very shortly after the operation. The third case 
was a child, two years of age, whose skull had evidently 
undergone a premature synostosis. It survived the opera- 
tion and showed a little improvement, inasmuch as it began 
to play with other children in the hospital ward, but dur- 
ing the four months after operation did not learn either 
to talk, stand, or to walk. The improvement was, in fact, 
very slight indeed. Wishing to give the method a further 
test, the child was operated upon a second time, and an 
opening similar to the first was made in the opposite half of 
the skull. The child died from " shock " four hours after 
the operation was finished. 

Figure 131 is inserted to show tne condition of the skull* 
after the second operation. It will be seen at first glance 
that the original opening that was made became very much 
smaller after four months ; that a tense fibrous tissue was 
formed over this opening, and that therefore four months 
after the initial operation the brain was not under condi- 
tions much more favorable than those which preceded the 

*This skull was demonstrated by Dr. A. Jacobi, at the International Congress, held 
in Rome, 1894. In the last ten years the author has not advised this operation. 



510 THE NERVOUS DISEASES OF CHILDREN. 

first operation. I would therefore conclude from my own 
experience * that Lannelongue's operation is not likely per- 
manently to increase the intra-cranial capacity, and further- 
more, that operations upon young children are extremely 




Fig. 131. — Skull of a Child Two Years of Age, showing (on the left of the figure) the 
Narrowing of the Opening Made According to Lannelongue's Method Four Months 
Previous to Death. Death ensued four hours after second operation. 

dangerous, the children often dying from shock, or from 
the loss of blood. 

If we wish to utilize Lannelongue's idea it will be better 
to make a circular .opening over that part of the brain 
which from the symptoms in the case would appear to be 
deficient. In children whose frontal lobes are stunted in 
growth, circular openings in the frontal or in the parietal 
bones would give a very large measure of relief, and I am 

* Rourneville, Jacobi and almost every one else have come to similar conclusions. 



DEFECTIVE DEVELOPMENT OF THE BRAIN. 51 1 

confident that if the operation is entirely aseptic cerebral 
hernia need not be feared. The bone should not be re- 
placed, but it will be well to provide such children with 
some form of external protection, or else the danger of di- 
rect injury to the brain from falls would be very great 
indeed. The author wishes once more to emphasize the 
fact that there are relatively few children in whom a small 
skull is the primary, or even the most important, cause of 
arrested development of the brain. The fault lies chiefly 
with the brain itself and cannot be remedied by operation. 

Partial Cerebral Defects. — In speaking of the con- 
dition of the brain in microcephalic children I have alluded 
to the fact that a part of the brain, and not necessarily the 
entire brain, may be checked in its development. This is 
particularly marked with regard to the frontal and the 
occipital lobes. These conditions deserve special atten- 
tion, moreover, because some of them are attended by 
symptoms which enable us to diagnosticate during life the 
exact area of defective development. It is fair to assume 
that if the frontal lobes are wanting, intelligence will suffer 
most, whereas the movements of the child will be normal, 
sensation will be undisturbed, and all the special senses, ex- 
cept possibly the olfactory sense, will be well developed. It 
is not claimed that intelligence is altogether the property of 
the frontal lobes, for the various functions that are so essen- 
tial to normal mental faculties are stored in the most diverse 
portions of the brain, and a defect in one or more of these 
sensory divisions of the brain in young children will natu- 
rally interfere with normal mental development. If the de- 
fect is in the motor areas, the disturbances of motion and of 
sensation will naturally point out the defective area. While 
porencephaly is extremely frequent in this region, I have 
not yet seen a single brain that exhibited normal develop- 
ment of its fissures and convolutions in all but the motor 
region. 

Defective Development of the Occipital Lobe. — 
Defective development of the occipital lobe, as indicated by 
an atrophy of the gyri of this lobe, is well illustrated by the 
case represented in Fig. 132, which has been reported by 
Peterson. " The patient was a female, twenty-eight years 



512 



THE NERVOUS DISEASES OF CHILDREN. 



of age, and was from birth an idiot, with epilepsy and left 
hemiplegia (slight) ; she was almost blind, being able only 
to distinguish light from darkness. Both occipital lobes 
were greatly atrophied ; the loss of tissue was made up for 
by the widely dilated posterior horns of the ventricles. 
The cerebellum projected far beyond the posterior lobes ; 




Fig. 132.*— Brain of a Woman Aged Twenty-eight years, Idiot from Birth and almost 
Blind. Defective development of both occipital lobes, which appear to be mere ap- 
pendages to the more normal anterior portions. Drawing made from specimen ; pia 
of left half not removed. 

the atrophy and sclerosis extended partly into the motor 
area of the right side." 

Defective development of the larger ganglia is not met 
with in cases in which there are no other large cerebral de- 
fects. This statement must be accepted with some reserve, 

* The author is indebted to Dr. Peterson for permission to have the specimen copied. 



DEFECTIVE DEVELOPMENT OF THE BRAIN. 5 1 3 

for little attention has been paid to this subject, and of the 
defective brains that have been examined the records say 
very little about the ganglionic region. 

The author has had occasion to diagnosticate defective 
development of the cerebellum. The diagnosis was made 
in a girl, eight years of age, born without difficulty and at 
full term. The child had never learned to walk or to talk. 
There was not a sign of spasticity or of actual paralysis. If 
the girl was placed on her feet she would fall over at once 
to the right. Equilibrium could not be maintained while 
the child was in the erect position. Sitting up in bed or on 
a table she was able to move the various parts of the body. 
The knee-jerks were absent ; hearing and sight normal. 
There was a condition of complete idiocy. The defect of 
the cerebellum was evidently part of a general brain defect. 
The case bore a superficial resemblance to Friedreich's dis- 
ease, but could be differentiated from it by the early onset 
and the development of idiocy very soon after birth. 

Agenesis Corticalis. — In the conditions previously 
described gross defects of the entire brain, or of parts of 
the brain, have been referred to. In the condition of agene- 
sis corticalis the size and weight of the brain may be en- 
tirely normal. The changes to be observed are those dis- 
covered on microscopical examination. The only outward 
indication of the imperfect development in the external 
configuration of the brain is abnormal fissuration. The 
fissure of Rolando dips straight into the fissure of Sylvius, 
without leaving a small convolution between the two fis- 
sures as in normal brains. (See Fig. 133.) The interparietal 
fissure extends farther back than normal into the occipital 
lobe; the island of Reil may be entirely exposed instead of 
being obscured from view by the margins of the fissure of 
Sylvius. Such differences as these are characteristic of a 
lower order of brain development (criminal brains, for in- 
stance). On minute examination of such brains that have 
been hardened, the large nerve-cells in the cortex are seen 
to be distorted, their contours not as well marked as in the 
normal brain, the protoplasmic contents are found in va- 
rious stages of disintegration. The nuclei are either sub- 
divided or destroyed, and the fine nerve -processes, so 



5H 



THE NERVOUS DISEASES OF CHILDREN. 



beautifully distinct in the normal cells, have disappeared 
entirely. Sections through the cortical tissue of any part 
of the hemispheres reveal practically the same condition. 
The anatomical study of two cases, made some years ago, 
led to the recognition of a special type of idiocy with blind- 
ness and ending in marasmus. (See section on Amaurotic 
Family Idiocy.) It has been definitely ascertained that the 
agenesis corticalis is part of a degeneration of the gray mat- 
ter of the entire central nervous system. 




Fig. 133 — Photograph of Brain of Author's First Case of Amaurotic Family Idiocy. 
The histological condition was described as an " agenesis corticalis." In the above 
figure, the confluence of fissures and the exposure of the Island of Reil are the signs 
of a low order of cerebral development. Through the hardening process the condi- 
tions have been accentuated a little, but they were present in the fresh specimen. 
At x, and in the frontal lobes, sections had been removed for histological examina- 
tion. Other letters refer to fissures. 



MACROCEPHALUS. 

This term may be applied equally well to an increase in the size of the 
head due to hydrocephalus, as to those rarer conditions in which an in- 
crease in the volume of the brain has brought about a corresponding in- 
crease in that of the skull. Hypertrophy of the brain is very rare indeed. 
While some maintain that the increased size of the brain is due to a prolif- 
eration of the neuroglia, others believe that it is the result of an increase 
in all the elementary structures of the brain, but the histological examination 
has been made in so few cases that the point has not been decided. Steffen 



DEFECTIVE DEVELOPMENT OF THE BRAIN. 5 1 5 

remarks correctly enough that if the increased size were due to a prolifera- 
tion of the neuroglia, this proliferation would come to a standstill and a par- 
tial atrophy and sclerosis would be the natural result, but in those brains 
which have been examined post mortem true hypertrophy was found and no 
indications of a sclerotic condition. Rilliet and Barthez, Gerhardt and others, 
evidently confound cases of interstitial encephalitis with hypertrophy of the 
brain. Steffen is correct in insisting that the term should be restricted to 
those in which there is a true hypertrophy of a part of, or of the whole brain. 
In consequence of the increased growth of the brain the resisting skull 
causes increased pressure ; as a result of this the blood-vessels are narrowed 
and the circulation is impaired, whence it follows that the brain is pale and 
anaemic ; the convolutions are often considerably flattened, and the fissures 
become more or less indistinct. The ventricles may be greatly compressed, 
or almost obliterated. By compression from without the cerebro-spinal fluid 
is driven largely out of the ventricles. As long as the fontanelles remain open 
the danger from compression is not as great as it is in later years when the 
entire skull represents an unyielding mass. As long as the fontanelles re- 
main open a true hypertrophy can be differentiated from a chronic hydro- 
cephalus. The hydrocephalus increases more rapidly as a rule than the 
hypertrophy does. The fontanelle will be more distinctly pulsating in hydro- 
cephalus than in the other condition. The form of the head will van' much 
in these two processes. In the case of an hypertrophied brain the entire 
skull will be equally enlarged ; whereas in hydrocephalus the accumulation 
of fluid in the anterior or the posterior horns of the ventricles tends to a special 
increase in the frontal and in the occipital portion of the skull. The sutures 
may be forced apart by the increase in fluid. 

The clinical symptoms of this condition are not very different from those 
found in chronic hydrocephalus ; a considerable mental development, or even 
normal mental development, is compatible with hypertrophy of the brain 
tissue, and if the fontanelles are not closed the increase in the size of the 
brain does not entail a very considerable increase of pressure, but it is false 
to suppose that children with hypertrophied brains would naturally be 
brighter than those whose brains are of normal dimensions. The hyper- 
trophy is self-limited by reason of the additional pressure it causes. 

If the size of the head increases very much, children are unable to carry 
the head, feel the need of supporting it, become apathetic, morose, and have 
a tendency to fall ; if the anaemia increases, convulsions, disturbances of vi- 
sion, vomiting, and a gradual cessation of all the faculties may precede death, 
which occurs after an indefinite period of time, unless brought about more 
rapidly by an intercurrent disease. It will not do to devote too much atten- 
tion to this condition, which is of extremely little practical value. The diag- 
nosis of hypertrophied brain will be made much more frequently at the post- 
mortem table than in the wards of a hospital or in private practice. 

Defective Development of the Cranial Nerve 
Nuclei should be considered in conjunction with the larger 



5 16 THE NERVOUS DISEASES OF CHILDREN. 

defects described above. These cases have received but 
very little attention, yet they are of great interest both 
from a clinical and from an anatomical stand-point. The 
nuclei most frequently affected are those connected with the 
nerves governing the ocular muscles. Cases of congenital 
ptosis, unilateral or bilateral, are so common that every 
one can recall persons so affected. Forms of congenital 
deficiencies in facial innervation come under this heading. 
Gowers has made a short note of them under the heading 
of " Infantile Oculo-facial Palsy." Moebius collected over 
forty cases of this class and reported upon them in the year 
1892 ; while Schapringer gave a most intelligent descrip- 
tion of a similar condition which he termed " Congenital 
Bilateral Pleuroplegia (Paralysis of Lateral Movements) 
and Facial Palsy." His case, which we shall give some- 
what more in detail, shows, however, that his title did not 
exhaust the clinical symptoms of the case, for there was evi- 
dence of involvement of the fifth nerve as well as of the sixth 
and seventh. I should, therefore, prefer to give to all these 
diseases the proper designation of " Congenital Nuclear 
Palsy." That there is in these conditions an actual defect 
in the development of the cranial nuclei there can be little 
doubt, and that these defects are associated with other con- 
ditions of defective development is proved by the occur- 
rence of these congenital nuclear palsies with imbecility or 
idiocy. (Oppenheim, Gowers, Heubner, and the author.) 
I have seen several interesting cases of congenital nuclear 
palsy in patients afflicted with scleroderma and myxcedema. 
We shall be justified, therefore, in relegating to this class 
of cases all those patients in whom there is a congenital 
defect of a unilateral or bilateral character implying insuffi- 
cient innervation of the muscles governed by any of the 
cranial nerves. The forms that are most frequently observed 
are congenital ptosis, ophthalmoplegia externa, partial or 
complete, facial paralysis, and paralysis of the tongue. A 
close clinical and anatomical relationship would seem to 
me to exist between these cases of congenital nuclear palsy, 
and cases of hereditary progressive muscular atrophy, pos- 
sibly also between these nuclear palsies and those with local 
muscular defects. 



DEFECTIVE DEVELOPMENT OF THE BRAIN. 



517 



The symptoms of these types will naturally vary very much. I propose 
giving a short summary of Schapringer's case, which exhibits a more complex 
association of symptoms than any other with which I have become acquainted. 
The history in brief is this : A girl, eight years of age (Fig. 134), who was 
suffering at the time from a slight bronchial trouble, was examined by Dr. 
Schapringer in 1889. The child was of normal development, and of average 
intelligence, although she was not sent to school, and had not yet learned 
to read. In walking she stumbled and fell more frequently than other chil- 
dren of the same age. She states that this is in consequence of a weakness 
in the legs, and not on account of the visual disturbance. Her face is pale, 
expressionless, and mask-like. The naso-labial fold is absent on both sides, 
there is not the slightest indication of 
folds in the integument of the face 
either in laughing or crying. Her 
laughing and crying sounds cannot 
easily be distinguished. She cannot 
close her lips. Substitutes Unguals for 
labials, " tata " for " papa," etc. The 
right angle of the mouth is drawn 
downward and outward. Whistling, 
blowing, etc., are impossible. She is 
not able to wrinkle the forehead either 
vertically or transversely. When her 
eyes are open the ordinary amount of 
eyeball is uncovered. Quite often one 
lid hangs a little lower than its fellow, 
but the eyes can be fully and com- 
pletely opened, proving that both leva- 
tores palpebrarum are not involved. 
Complete closure of the eyes is impos- 
sible, although the lids can upon forci- 
ble effort be closely approximated to 

one another. There is a distinct epicanthus, the caruncle and plica semilu- 
naris are only slightly developed on both sides. 

Ordinarily the axes of the eyes are parallel, frequently, however, the right 
eye is directed a little more upward and slightly outward. She is able to 
follow an object with both eyes if it be lowered or elevated in the median 
line. If the object be approached to her face, keeping it in the saggital plane 
she follows it readily, and she will keep her eyes fixed upon a finger held 
near the nose for a long period without fatigue. These, however, are all the 
movements of which the eyeball is capable. The two internal recti contract 
freely if required in order to bring the eyes into convergence for near objects. 
If, however, one of these muscles be required to act in unison with the ex- 
ternal rectus of the opposite eye for a conjugate lateral vision, there will be 
found an utter inability to do so. The two external recti muscles are either 
completely paralyzed or very deficient. The internal recti are not wholly 
paralyzed. When the child wishes to see an object situated to the side 




Fig. 134.— Girl, Eight Years Old, with 
Congenital Pleuroplegia. ( Scha- 
pringer.) 



5 18 THE NERVOUS DISEASES OF CHILDREN. 

of the median plane, instead of rotating the eyes she turns her entire head. 
The fundus appears normal, except that the vessels are a little more 
tortuous than usual. The functions of the ciliary muscles are normal. 
There is no strabismus convergens. The size and mobility of the pupils 
are normal. When the tongue is protruded it appears to the left of the me- 
dian line ; the left half is a trifle smaller than the right. When eating, the 
child is obliged to use its finger to dislodge food from the cheeks. She is 
unable to masticate hard substances, such as crusts. She can move the 
lower jaw laterally toward the right side, but not toward the left ; thus indi- 
cating a paralysis of the right pterygoid muscle. She has uvula bifida. There 
is an hypertrophied condition of the canine ligament of the right side, and 
normal development of the left. In addition to these congenital defects the 
author mentions a deflection of the distal phalanges of the index-fingers at an 
angle of about one hundred and fifty degrees in the direction of the middle 
finger. He interprets this also to be a congenital abnormality. The child has 
the condition known as " funnel chest." 

Reviewing the symptoms, which I have quoted very 
freely from Dr. Schapringer's paper, it is evident that the 
congenital defects involve the motor branch of the fifth, the 
hypoglossal, and the facial nerve, also the tracts which gov- 
ern the conjugate lateral movements, viz., the third and 
sixth nerve nuclei, and the nerve-tracts connecting the 
same. The only criticism to be passed upon this interest- 
ing case of Dr. Schapringer's is that while he has invented 
a good term for the paralysis of the lateral movements, 
the case presents other symptoms; and that the congeni- 
tal bilateral pleuroplegia is the most prominent symptom 
of the condition which we might define in an impartial 
way as one of bilateral congenital nuclear palsy. Cases of 
this description are evidently rare, for Schapringer had been 
able to collect but four others like the one he described. 

During the past ten years a number of authors have 
reported instances of congenital unilateral facial palsy, pre- 
sumably due to defective development of the nucleus. 
Schultze described this condition in a girl of five years, 
Bernhardt in a man of twenty-four years; and Remak has 
given an account of a young man of eighteen years, who 
presented a congenital defect of the platysma myoides, 
associated with slight bilateral ptosis, and a limitation of 
ocular movements upward. Surgical treatment only is 
worth considering. 



DISEASES OF THE MIND. 

CHAPTER XXIX. 

INSANITY. 

In the preceding chapters we have discussed the or- 
ganic diseases of the brain, and have touched upon con- 
ditions due to arrest of development and to morbid affec- 
tions of the central nervous system during the period of early 
growth. Mental disorders may be similarly subdivided. 
Idiocy represents the psychic derangement due to arrested 
development; it is so distinct and so large a subject that it 
will be treated separately. Insanity includes the morbid 
conditions coming on after a fair degree of mental develop- 
ment has been reached, and constitutes the subject-matter 
of this chapter. 

Insanity in the child resembles closely that in the adult. Such differ- 
ences as do exist are dependent upon the unusual sensitiveness and irritabil- 
ity of the youthful mind, and upon the paucity of concepts which have been 
formed prior to the development of the insanity. At a very early day sensory 
impressions become fixed ; hallucinations are possible, therefore, in very young- 
children. But the normal mind does not develop systematized concepts nor 
the logical faculty during the first few years of life, and prior to this stage of 
mental development delusions — faulty, unfounded beliefs— cannot exist. The 
delusions of an insane child will differ from those of the adult in view of the 
limited ideas it possessed during the period of mental health. Its delusions 
will be concerned largely with its own individuality and its relation to the 
family, its teacher, and its God ; but the delusions of the adult in regard to 
his wealth, his position in the religious, social, and political world, are entirely 
foreign to the child's mind. 

The normal child lacks, moreover, the judgment and the power of self- 
restraint by which the adult is guided and controlled in his actions. Mey- 
nert has charmingly depicted the egotism of the child, and the constant 
struggle of the healthy individual to rid himself of the ideas of self until he 



520 THE NERVOUS DISEASES OF CHILDREN. 

is enabled to sacrifice his individuality and his entire being for the good of 
the family, the state, and of all mankind. The child for a long time remains 
upon that lower plane in which passion and the gratification of desires are 
the mainspring of all action. 

The experience of centuries has taught us that by gradual stages, and 
through the influence of training and education, the selfish views of the 
child can be transformed into the more generous views of the adult. This 
process of evolution may be checked by disease ; it is only by comparison 
with the views and attainments of children of the same age that we can es- 
tablish the standard to which every child should conform. 

Insanity in childhood is relatively rare. According to 
the statistics of Hagen (quoted by Spitzka) only one in 
70,684 children annually becomes insane, excluding those 
born so. Spitzka states that " over four per cent, of 3,244 
adult recorded private cases may be justly regarded as 
having been insane in childhood, while but twelve patients 
afflicted with infantile forms of insanity " were observed by 
him during their infancy. From this it is evident that we 
must not only regard the full-fledged insanities of early 
life, but also those morbid conditions in early years which 
foreshadow the outbreak of mental disease in later years. 

The psychoses are not developed in very young infants, 
although Greding reports a child that was a " raving 
maniac " at birth. The story must be taken with a grain of 
salt. Rush has noted the occurrence of " mania " at the 
age of two years, and Sinkler has observed it in children 
three years old. 

After the period of first dentition is past, faulty psychic 
inheritance may become noticeable ; but the age of puberty 
is the one fraught with greatest danger. The author had 
under observation various members of a family, all of whom 
(two boys and three girls) have developed mental disease 
between the ages of twelve and fifteen years. All classes 
of the socia 1 body seem to be equally prone to mental dis- 
ease ; while idiocy is unquestionably more frequent in the 
poorer classes than among the well-to-do. 

Etiology. — Heredity is the potent factor in the cau- 
sation of juvenile and adult insanity. There is always the 
danger of over-estimating the importance of any one factor, 
but the daily experience of alienists and neurologists helps 
to enforce this belief. The importance of heredity is im- 



INSANITY. 521 

pressed upon us, too, by the different course of acquired 
insanity in persons with or without an hereditary taint. 
Psychic disorders constitute, as a rule, the last in a series 
of degenerative diseases ; neurasthenia, chorea, epilepsy in 
the parent may lead to insanity or idiocy in the child. By 
way of exception healthy individuals may emanate from 
families with a distinct neurotic or psychic taint. Mental 
derangement in children is ordinarily developed, if one or 
both parents have been insane, if one or both have been 
afflicted with a severe neurosis, if one or both have been 
addicted to alcohol, and above all, if the parents are related, 
and belong to a family in which nervous or mental disease 
has been of common occurrence. Individuals of different 
but tainted families will also be liable to generate insane 
children, or such as show distinct stigmata of degeneration 
(deformities of the skull, of the. ears, gothic palate, stunted 
growth, abnormal sexual appetite, early masturbation, and 
the like). Closely allied to the hereditary forms of juvenile 
insanity are those due to traumatism during birth. Meyer 
has insisted on this. The author has seen innumerable 
cases of idiocy due to this cause, but not a single instance 
of any other form of mental derangement. 

Arrested physical growth is often associated with men- 
tal impairment due to hereditary causes. A girl, aged 
twenty, whom I have seen in repeated maniacal attacks, 
is stunted in growth, being only as tall as a child of 
twelve, without any sign of mammary development or of 
menstruation. The girl stutters a little, but is fairly bright 
in the interval between the maniacal attacks. 

Among the causes of insanity acquired in early child- 
hood, the chief one is beyond doubt traumatism to the skull. 
Emminghaus has found this factor in fourteen of one hun- 
dred and three cases, not including those in which the injury 
was followed by epilepsy and mental derangement. Loss 
of memory and complete dementia, or maniacal excitement, 
are the forms of insanity commonly developed from this 
cause. The occurrence of idiocy after relatively slight in- 
juries need not be insisted on in this connection. Menin- 
gitis early in life, ear disease, insolation, epilepsy, hysteria, 
chorea, severe emotional excitement, grief, shame, disease of 



522 THE NERVOUS DISEASES OF CHILDREN. 

the heart, intestinal parasites, acute infectious diseases are 
the conditions which predispose to the development of in- 
sanity. To these might be added excessive work at school, 
maltreatment at home, starvation, and cruel punishments. 

Masturbation is another and a potent cause. The impor- 
tance of this factor has been underrated by some ; it can 
scarcely be overrated. There is a just difference of opinion, 
however, as to the influence it exerts in generating mental 
disease. Many claim that it is often the early symptom of 
an insane condition. There can be no doubt of this, but it 
is equally certain that the continuance of the habit brings 
about a rapid deterioration of the individual's mental state. 
It is often acquired by imitation, and quite as often it would 
seem to be the result of natural tendencies. In the former 
class the practice may occasionally be checked ; in the lat- 
ter, the habit is continued, both in boys and girls, in spite 
of the most persistent watching. The more stubborn cases 
are generally those characterized by other distinct signs of 
degeneration. The evil effects of the vice become most 
pronounced at the time when the individual is subjected to 
the first serious strain. Several of such young patients 
have done well at school, have passed their examinations 
successfully, but have broken down completely in the firrt 
competition with boys of mature and normal intellects. 

FORMS OF INSANITY. 

A detailed account of all the symptoms of insanity 
would far exceed the limits of this treatise. By combining 
a discussion of the symptoms with a description of the types 
of mental derangement, as they occur in early life, we shall 
be able to give a sufficient survey of the entire subject. It 
is best to begin with those symptoms and conditions which 
are on the border-line between sanity and insanity. 

Imperative Concepts. — The orderly sequence of thought 
in the child and in the adult may be interrupted by foreign 
and recurrent concepts, which obtrude themselves upon 
the individual's consciousness, which annoy him and tyran- 
nize over him, but of which he is not able, at least for a 
time, to rid himself. 



INSANITY. 523 

TJicse imperative or insistent concepts are very common in 
childhood ; at times they disappear without leaving any 
trace of mental defect behind them ; in some children they 
represent the first stages of chronic forms of insanity. 
Imperative conceptions may lead to imperative actions, to 
morbid fears, and morbid impulses. 

Many normal persons have passed through physiological states akin to 
these imperative concepts. On going to bed the thought arises suddenly 
that the light has not been properly turned down, that the gas might be 
escaping. They assure themselves that all is well, and after a few minutes 
the same fear or concept comes upon them again. The person so troubled 
may attempt to allay his fears by methods which ordinarily carry conviction, 
but in spite of himself the same insistent idea annoys him. Returning to 
one's door again and again to make sure that it is properly locked ; the an- 
noying thought that the letter which has just been mailed was not properly 
signed or sealed ; such are some of the imperative concepts of daily life. 
The sight of a knife may suggest the harm that can be done with it ; a per- 
son standing on a precipice or on a high tower has a feeling as though he 
would be dragged into the depths below. The child, after it has passed the 
first five or six years of life, may have similar experiences. It is often tort- 
ured by these insistent concepts. Some pupils at school have to do every- 
thing over and over again ; they never finish a sum in arithmetic as quickly as 
others because the doubt arises whether they have done it properly, and they 
are impelled to do everything a definite number of times (arithmomania). As 
a rule, they can assign no reason for this. A little patient would never ad- 
dress any one without repeating the name twice ; he was conscious of this 
peculiarity, was finally induced to repeat the name a second time to himself 
in such a low tone of voice that no one could hear it, and gradually lost the 
habit. 

Another lad, well known to the author, would do everything three times 
for fear of having done it twice. He would take three steps at a time in going 
upstairs ; if there were but two he would jump back from the first and then 
walk up the two, so as to make three in all ; before going to bed he touched 
the floor three times ; as soon as he was in bed he would get up again to 
make sure that he did not touch it twice. These concepts and actions were 
imperative, but they were developed from a fear that was engendered in him 
at the time of his father's death that his mother might be the second one to 
die. This caused him to avoid " two " in every way. After a number of 
years the imperative concepts were overcome. 

Closely allied to these conditions are the fears of con- 
tamination (the mysophobia of Hammond ; delire du 
toucher of Legrand du Saulle). I have known several 
children who would not touch the knob of a door, or take 



524 THE NERVOUS DISEASES OF CHILD RE A. 

anything out of other people's hands. One child would 
not be washed because the water flowed through pipes and 
the pipes were not clean. The fear of open places (agora- 
phobia of Westphal) occurs in children, though not nearly 
so often as in adults. Some years ago the author treated a 
young girl, twelve years of age, who could not be induced 
to come to his office because she would have to cross a 
small hill over a tunnel ; she was certain that she could 
never get across. These fears may increase in such num- 
ber as to give rise to what Mills calls pantophobia. 

Children as well as adults are troubled by insistent ques- 
tions — generally abstract ones — which interrupt their trains 
of thought. Why is the sky blue, the water wet, the ball 
round ? Occasionally they soar into the field of religion. 
Why is God just? and so on. 

All these conditions may be of slight significance unless 
they lead to imperative acts of violence (cutting a child's 
throat at the sight of a knife, etc.) ; or unless the effort to 
suppress these morbid concepts and impulses produces great 
excitement. A little patient of mine, years ago, was so 
annoyed that he would every now and then pass into a 
maniacal state or entertain suicidal ideas. Westphal was of 
the opinion that these imperative conceptions were never 
developed into delusions ; this is true in general, but they 
are often present in persons who develop systematized 
delusions suggested by the contents of some one imperative 
conception. Imperative concepts and impulses are very 
apt to occur in children who have passed through some ex- 
hausting disease, or some intense emotional excitement. As 
children grow older they may learn to disregard annoying 
concepts, and thus rid themselves of them. 

Cerebral Neurasthenia. — Nervous exhaustion, or 
neurasthenia, is, on the whole, so rare in children that we 
have not devoted any special chapter to the discussion of 
this disease. The causes which give rise to neurasthenia in 
the adult are far less potent in childhood. Excitement, 
over-work, cares in early years, are more apt to produce 
hysterical and choreic conditions than neurasthenia ; occa- 
sionally, however, the cerebral type is developed in young 
persons between the ages of ten and fifteen years or older. 



INSANITY. 525 

The chief symptoms are, inability to concentrate the atten- 
tion upon any special work, or excessive restlessness follow- 
ing upon such an effort. A feeling of pressure upon the 
top of the head, a dragging sensation over the nape of the 
neck and the upper portion of the spine, general irritability, 
a morose disposition, sleeplessness and a feeling of fatigue, 
a slight increase in the reflexes, sensations of heat and cold 
complete the details of this condition. 

Cerebral neurasthenia occurs mainly in the children of 
hysterical or excessively neurotic parents, and in those who 
come of healthy parentage but are pushed inordinately in 
their work at school to satisfy the demands of ambitious 
parents. It has been the custom to attach the entire blame 
to present educational methods, but the chief fault lies with 
the father or mother who cannot recognize the child's in- 
ability to cope with other children of the same age. Boys 
or girls who recognize that they are at a disadvantage in 
competition with others become discouraged and moody, 
and often develop a condition of melancholy depression. 

Some years since, a young girl, aged thirteen, was brought to me, who had 
just entered upon the work of a high-school in which an older sister had 
graduated with high honors. The patient was told by her mother that she 
would be a discredit to her family unless she did at least as well as the older 
sister had done. The child made a strenuous effort to come up to these 
expectations, but the result was evident on examination. The child com- 
plained of intense headaches, frontal and vertical, of a feeling as though 
she were being crushed under a weight. She looked pale and haggard 
in consequence of the loss of sleep. Her appetite had left her, and for a few 
days before she was examined was either in a drowsy condition or else 
would cry and complain of her misfortune. She knew that she could not 
possibly do satisfactory work at school, and had developed the idea that she 
was thoroughly unworthy of the care which her parents had bestowed upon 
her. 

The child was taken out of school at once, sent to some relatives in the 
country, and was told that she would get entirely well provided she would 
not think of school, or anything connected with school matters, for a period of 
several months. The parents were also instructed never again to force the 
child's work, or to foster any inordinate ambition in her. The older sister 
was a bright, strong, well-developed girl, who could in all probability have 
stood any amount of work without showing signs of fatigue. The younger 
child was less well developed physically, and presented the remnants of 
former internal hydrocephalus (a bulging forehead and very large transverse 



526 ' THE NERVOUS DISEASES OF CHILDREN. 

occipital diameter). Had the parents been allowed to continue in their foolish 
course much longer, this child, who presented on the whole none but neuras- 
thenic symptoms, would unquestionably have developed a serious form of 
psychosis. 

The course of cerebral neurasthenia may cover a period 
of several weeks or months, according to the time at which 
a radical effort is begun to check the development of the 
disease ; but the affection is an eminently curable one. The 
condition should be treated very much as is the same disease 
in the adult. The most important point is to give body and 
mind complete rest, to take the child from school and to 
prohibit all mental fatigue either in the way of school or of 
home studies. If a proper arrangement can be made, it 
is best to place the child under care of a sensible nurse, and 
subject it to the rest-cure. A separation from hysterical 
and irritating relatives is absolutely essential. Careful feed- 
ing and the ordinary tonics — above all, iron, arsenic, and 
small doses of strychnia — will help to bring about a rapid 
improvement. After four to six weeks the child should be 
allowed to roam about in the country, and its mind should 
at least for a year or more be kept entirely free from every 
sense of duty and obligation. 

Hypochondriasis is often associated with neurasthenia, 
and occasionally it is a primary affection. The fear of in- 
sanity is not as pronounced as in adult neurasthenics, but 
the fear of death is common. The uncomfortable sensations 
in the head frighten the child, and soon it takes notice of 
every little symptom to which other children would pay 
no attention. In such introspection children are encour- 
aged by over-anxious mothers. Like the adult, the young 
hypochondriac hears his heart palpitate, takes notice of the 
rumbling of his bowels, watches his skin most carefully, 
and exaggerates the importance of every pimple that ap- 
pears upon it. At the age of puberty a boy's attention is 
rivetted easily upon the sexual organs. He may be fright- 
ened by the occurrence of erections or by the difference in 
the size of the testicles. A bright lad, aged fourteen, whom 
I treated for a long time, and who came of a highly neu- 
rotic family, was at great pains to prove to me that his 
testicles were " detached " from the rest of his body, be- 



INSANITY, 527 

cause he could move them about freely. Early masturbation 
intensifies such fears. Westphal reports the history of a 
young hypochondriac who complained of abnormal sen- 
sations in the head, in the feet, in the abdomen ; his tears 
had been dried up, and all the mucus in the lungs had been 
expectorated. The boy's hypochondriacal mood was inter- 
rupted by a mild attack of maniacal excitement. The prog- 
nosis of hypochondriasis in the young is entirely favorable 
unless it is based upon a marked hereditary taint. The cure 
of the condition lies in sensible management of the youth's 
mode of life, in diverting his mind from himself by moder- 
ate indulgence in out-door sports, by providing him with 
the companionship of sober-minded lads of the same age. 
I have known boys suffering from sexual hypochondriasis 
to be forced by parents into early intercourse with the 
opposite sex. The procedure is invariably harmful. 

The preceding forms of mental derangement are less 
serious than those which we must now consider. 

Mania. — The term " mania " denotes a form of mental 
disease in which there is a marked acceleration of all cere- 
bral and physical functions, generally associated with a 
feeling of well-being. In children this condition is rela- 
tively rare ; it is often confounded with a temporary active 
delirium, which comes on after the ordinary febrile diseases 
of childhood. In true mania restraint is entirely removed ; 
the patient's ability and desire to do anything and every- 
thing he chooses knows no bounds. Thoughts and impulses 
follow rapidly upon one another. Emminghaus has well 
said that an exalted mood, a rapid succession of incomplete 
thoughts, unbounded desire to make everything it sees its 
own, are characteristic of the healthy child. But the normal 
child, under the restraining influences of education and train- 
ing, soon learns to curb its desires and to develop an orderly 
train of thought. The young maniac has apparently cast 
aside all restraint and gives himself up completely to his 
passions and his impulses. His incessant activity leads him 
to tear and destroy everything within his reach ; he is cruel 
to others and does not hesitate to inflict injury upon him- 
self. There is no trace of a sense of decency or propriety, 
even young children using the foulest language. Immature 



528 THE NERVOUS DISEASES OF CHILDREN. 

thoughts follow rapidly upon one another ; everything the 
maniac hears amuses him ; a word suggests another that 
rhymes with it ; his answers are often quick, sometimes 
bright ; but they are flashes merely and do not denote real 
intelligence. 

The excessive restlessness and the rapid flight of ideas 
are not followed by a feeling of fatigue, which would be 
natural in a normal person. Loss of appetite and of sleep 
contribute to the exhaustion which is developed if the con- 
dition lasts for a number of weeks or months. 

The symptoms of mania come on in an insidious 
fashion, or may be preceded by a period of depression. At 
first the greater liveliness and activity of the child are sup- 
posed to be a mere exaggeration of its normal state ; by 
degrees the increase of all the symptoms points to a morbid 
state ; after the acme of the disease has been reached the 
condition remains unchanged for some weeks, and then the 
excitement gradually diminishes. A return of sleep is, as a 
rule, the first favorable sign pointing toward recovery. Re- 
current mania (several attacks and remissions) has been 
described. The exact duration of a maniacal attack may 
vary between five months and a year, but the state of in- 
tense excitement is, as a rule, much shorter. 

Among the etiological factors the acute febrile condi- 
tions and severe strain or emotional excitement are the 
most potent. The period of beginning menstruation is 
fraught with the greatest danger. The only cases of true 
mania which I have seen were in girls at this period, and in 
a boy, aged fifteen. One of the girls had passed through 
a series of examinations at school with great credit to her- 
self. On the morning following the last examination she 
refused to get out of bed, would not take her food, and 
would not allow herself to be washed. To all questions why 
she would not do as asked, she mumbled a few inaudible 
words ; soon she became wholly silent, and for a period of four 
weeks she lay in an absolutely stuporous condition, passing 
urine and faeces into the bed, and refusing to take nourish- 
ment. From this condition she passed quite suddenly into 
one of most violent mania, in which she tore everything 
that she could lay hands on, sang and cursed all day long, 



INSANITY. 529 

spat at every one, tore her own clothes into shreds, and 
would expose her person before every one. (In health she 
was a sweet well-behaved child.) The state of maniacal 
excitement lasted four months, during which time she lost a 
great deal of flesh. Six months after the first symptoms had 
appeared, menstruation set in, and from that time onward 
a rapid and complete recovery ensued. In other cases I 
have observed a development of mania after the cessation 
of the menses that had appeared a few times. A close 
relation between the menstrual flow and these psychic con- 
ditions cannot be doubted. 

While the prognosis is favorable, careful treatment is 
necessary in all cases. These children must be guarded by 
competent nurses ; mechanical restraint cannot always be 
avoided, but it should not be more forcible than necessary. 
Such patients are generally fit subjects for an asylum, and 
often do better there than at home, where greater restraint 
has to be applied. The author has treated such patients, 
however, in their homes by placing them in large, well-venti- 
lated rooms, away from the rest of the family and under 
the charge of two or more nurses. 

In home and asylum treatment, the hydrobromate of 
hyoscin (one two-hundredth to one one-hundredth grain 
three times daily), given by the mouth or hypodermically, 
is a valuable drug. In addition, sulfonal, trional or veronal, 
in moderate doses, should be given at night. Bromides are 
entirely useless, and opiates of little benefit. Prolonged full 
baths, followed by slightly cooler ablutions of the spine 
tend to calm the excitement, and sometimes help to induce 
sleep when all other measures fail. 

Melancholia represents a distinct form of insanity 
which has been observed in children, particularly between 
the ages of eight and fifteen years. It is not to be con- 
founded with simple melancholy depression, which may 
accompany many other morbid mental states. It is as nat- 
ural for a lunatic who supposes himself to be the victim of 
persecution, or a target for the raillery of others, to be de- 
pressed and melancholy as it would be for a sane person 
who found that all his efforts to succeed were in vain, and 
that everything and everybody were against him. 



530 THE NERVOUS DISEASES OF CHILDREN. 

In true melancholia the child is depressed without 
cause. Experiences which would please others make no 
impression upon its saddened mind. It does not care to 
play with other children, whose frolicsome ways are a 
source of annoyance. Games, books, theatres, have no 
charm for the melancholy child ; it seeks seclusion, sits in 
a corner by itself all day long ; will not speak spontane- 
ously, and, if spoken to, either does not answer at all or 
replies in monosyllables and with much hesitation. No 
reason for its moodiness is given ; after much questioning 
it may say that it feels sad, but does not know why. To 
the questions put to a little patient I received the answer, 
" I know, I know," accompanied by a nodding of the head. 
As it was recovering, the child stated that she knew every- 
one was against her ; that her parents did not care for her ; 
that she was to be punished because she had not loved 
them enough, and because she was so ugly (as a matter of 
fact she was good-looking). As this same child was taken 
to the country to hasten convalescence, she said : " I know, 
I know, you will burn me to ashes." 

In contradiction to the acceleration of all mental proc- 
esses in mania there is a distinct inhibition, a " slowing 
up " of all mental and physical processes in melancholia. 
Loss of appetite, restless sleep, slowness of all muscular 
efforts, obstinate constipation are characteristic features of 
this disorder. The child may be able to reason a little re- 
garding its unfortunate condition, and self-accusations are 
abundant. It accuses itself of unkindness toward others, 
of want of respect and love for its elders ; if it has had any 
religious training it develops the ideas of the " unpardon- 
able sin," and of lack of devotion to God — ideas which 
play a prominent part in the melancholy of adults. Hallu- 
cinations in keeping with the depressed mood, the sight 
of the devil, of a cruel teacher armed with all sorts of 
weapons of torture, may increase the depression, or else 
lead to a condition of excitement or frenzy (melancholia 
agitata). Unlike the maniac, the melancholy patient di- 
rects his frenzied impulses toward his own person. Self- 
mutilation is common. A young girl, aged thirteen, had 
to be restrained because she would insist upon tear- 



INSANITY. 531 

ing out her hair and attempted to cut deep into her 
skin. 

Suicide of children is frequently the result of melancholia. Like the 
adult, the child seeks to put an end to a life that is so full of trouble and 
misery. Statistics as to the frequency of suicide in children are rather unsatis- 
factory ; Emminghaus quotes those of Morselli that show the number to be 
greatest in Denmark and Prussia, and smallest in Belgium and Italy. The 
author has seen no statistics bearing upon America. In the majority of 
cases some mental disease is the cause of suicide in the young, and none is 
more frequent than melancholy. Some suicidal attempts are due to trifling 
causes — fear of punishment, chagrin over an unmerited rebuke, etc. 

Melancholia with stupor (melancholia attonita) is a more 
extreme form, which is either developed primarily or fol- 
lows upon a condition of simple melancholy. In this form 
the child lies in bed absolutely motionless, takes no notice 
of its surroundings, and cannnot be induced to smile or to 
say a single word. It reacts but feebly to every form of 
cutaneous stimulation ; it will to a certain degree tolerate 
pain rather than move. If passive movement is attempted, 
the limbs will retain the position given them, or the child 
offers considerable resistance. Urine and faeces are passed 
into the bed. During this condition of stupor disagreeable 
hallucinations and terrorizing delusions add to the child's 
misery. 

In the author's experience melancholia is the most fre- 
quent form of mental derangement in childhood. Its 
course varies very much. Some patients get well in a few 
months, others do not recover for more than a year. In a 
number of instances a condition of mania follows upon the 
period of depression. The prognosis is favorable as regards 
ultimate recovery ; it is certain that sixty per cent, of 
young melancholy subjects get well. As a rule, the pros- 
pects of early recovery are better in the agitated form, and 
less bright in the stuporous form. Melancholy may ter- 
minate in dementia, or in death from exhaustion or from 
suicide.* 

The treatment of melancholia is very simple. One or 

* The author has not entered upon an enumeration of the morbid anatomy of 
melancholia or mania, as there is nothing but pure hypothesis to proceed upon. The 
vascular theories of Meynert have not been corroborated by others. 



532 THE NERVOUS DISEASES OF CHILDREN'. 

more competent nurses must be deputed to watch over the 
child and protect it against all harm. It must be fed care- 
fully with a spoon, and will do best on milk, some cereals, 
scraped meat, and eggs. The stomach-tube should not be 
resorted to unless absolutely necessary. Opium in small 
doses, or hyoscyamia in the agitated forms, are the most use- 
ful drugs ; both can be given hypodermically if necessary, 
but they should be discontinued as soon as practicable. Sul- 
fonal, chloralamid, or trional will help to induce sleep and to 
reduce mental excitement. As in cases of mania, a warm, 
full bath is a valuable aid in treatment. 

There is no sufficient reason to remove such children 
from comfortable homes, if isolation at home is possible ; 
but if the environment of the child is an unfavorable one, 
the sooner it is taken to an asylum the better it will be. 

Periodic and Circular Insanity are very exceptional 
occurrences in children. The youngest patient of this class 
the author has seen was a boy, aged eighteen, and all of the 
patients described by Krafft-Ebing, Jacobi, Kelp, and others 
have been near or beyond the age of puberty. Periodic 
insanity consists of successive attacks of mania or of melan- 
cholia, followed by a lucid interval of months or years, and 
then a recurrence of the same conditions. Circular insanity 
is closely allied to this form. As described by Krafft-Ebing 
it is distinguished from the ordinary periodic insanity by 
an alternation of maniacal and melancholy stages followed 
by a lucid interval, and then a recurrence of derangement 
in the same or the reverse order as before. The diagnosis 
of circular insanity can be suspected, but not made, until 
the patient has passed through several cycles. 

The duration of each cycle may vary from several days 
to several weeks ; but in the author's experience as the dis- 
ease progresses the lucid periods grow shorter and shorter. 
The perodic and circular forms of insanity are of the herid- 
itary degenerative type, and the prognosis in them is far 
less favorable than it would be in ordinary mania or melan- 
cholia. During the maniacal or melancholy state the treat- 
ment would be similar to that described for each con- 
dition. These patients are dangerous to themselves and 
their surroundings, as they pass quickly from one stage to 



INSANITY. 533 

another. As soon as the circular and periodic character of 
the disease is established, it is best to keep such children 
under the constant observation of an attendant, or else to 
place them in special institutions. 

The author has not departed from the above account as given in the first 
edition of this book, although he is well aware of the changes which have 
been brought about in the classification of early mental disorders through the 
writings of Kraepelin and his followers, who have substituted for periodic 
and circular insanity the conception of a manic-depressive form of insanity. 
With the exception of the involution melancholias, Kraepelin would con- 
sider all periodic or recurrent forms of mania and melancholia to be merely 
different manifestations of a single psychosis, viz., manic-depressive insanity. 
Startling and revolutionary as Kraepelin 's doctrines may appear to be, the 
writer's ample experience would lead him to indorse this view, at least in part, 
but he must also insist that many a youthful individual may pass through 
periods of maniacal excitement or of depression without such an individual 
necessarily being a victim of a circular or manic-depressive psychosis. 

Dementia precox : Following Kraepelin's suggestions, alienists and 
neurologists have adopted this unfortunate term, designating thereby an entire 
series of youthful or adolescent forms of insanity characterized by a distinct 
tendency to progressive deterioration of mind. French authors, the writer, 
Dercum, and others have been at some pains to protest against the too liberal 
use of this term and against the practice of labeling almost every form of 
youthful insanity as a dementia praecox, for many of these patients never 
develop a condition of dementia and should be spared the stigma of a name 
that implies an incurable malady. On the other hand, it is well to admit that 
mental enfeeblement is a frequent accompaniment of insanity in early life. 

Dementia Praecox includes three distinct forms, each one of which may be 
developed at the age of puberty or even earlier : (i) Hebephrenia ; (2) Kata- 
tonia ; (3) the Paranoid form of Dementia Praecox. 

Hebephrenia as described by Kahlbaum and Hecker is characterized by a 
period of depression, with hallucinations and delusions of a corresponding 
type ; the patients are often conscious of their altered mental state and recog- 
nize their inability to vie with others of their age at school or even in sport ; 
they lack the power of concentration and of attention. Their minds are 
easily diverted from the subject in hand, their memory and judgment become 
defective, they are indifferent to family and friends. Volubility of speech may 
for a time conceal a real mental defect. The patients laugh or cry without 
reason, are apt to repeat high-sounding phrases, their letters are verbose and 
meaningless, their conduct and manners become affected, silly, theatric, and 
their moral sense may be more or less impaired. According to Kraepelin's sta- 
tistics only 8 per cent, of hebephrenic patients have any chances of recovery. 

Katatonia, first described by Kahlbaum, is characterized by states of 
stupor or excitement associated with conditions of " negativism," of muscular 
tension, of impulsiveness, with a tendency to automatic and stereotyped ac- 



534 THE NERVOUS DISEASES OF CHILDREN. 

tions, and with automatic repetition of words. All these peculiar mental 
conditions lead to distinct deterioration within a reasonably short period of 
time. 

The paranoid form of dementia prascox is characterized by the develop- 
ment of an entire series of extravagant, absurd and ever changing delusions, 
coming on frequently with an initial period of depression and leading within 
a few years to a condition of undoubted dementia. Its onset early in life and 
the rapid development of a dementia will help to distinguish it from true 
paranoia. 

If we accept the Kraepelin doctrines in whole or in part, the prognosis of 
these forms must be given in very guarded fashion. But there seems, to the 
writer, to be sufficient doubt in many of these cases as to the ultimate out- 
come to make it incumbent upon the physician to urge that such individuals 
be separated from the family at once, that they be given for a time, at least, 
wholesome employment in the country or elsewhere, and that they should not 
be made asylum inmates until they have proved either that they are incapable 
of conducting themselves properly when at large, or that their psychic condi- 
tion is such that they need institution treatment for their own protection and 
for the improvement of their symptoms. 

Paranoia.* — Primary insanity is a degenerative psy- 
chosis of the hereditary order characterized by hallucina- 
tions and delusions. The latter are primary symptoms and 
not secondary to the exalted or depressed mood, as in 
mania and melancholia. The delusions or " fixed ideas," be- 
come systematized, and dominate the mental activity of the 
individual to such a degree that they become the mainspring 
of all action. The paranoiac is not amenable to reasoning ; 
and his delusions cannot be dislodged by argument, as 
happens with the temporary delusions of the sane. He 
may have one set of delusions or many. The persistence of 
one or many proves that the entire logical apparatus is out 
of gear. It is wrong to claim that any person is insane on 
one point only ; he may show his insanity in one direction 
chiefly, but his mental derangement is as marked as though 
he had dozens of fixed ideas. 

The systematized delusions of paranoia may be divided 
into two great groups ; first, delusions of persecution ; and 
secondly, delusions of grandeur ; the latter may be sub- 

* The writings of Snell, Westphal, Sander, Krafft-Ebing, and Meynert, have con- 
tributed most to an understanding of this subject. Spitzka treats the subject very 
lucidly under the name " Monomania,"' preserving an old term, but discarding the doo 
trine of the " Monomanias " which did so much harm in psychiatry. 



INSANITY. 535 

divided into the religious, the political, and the erotic type. 
In this form of insanity, with delusions of persecution, the 
patient believes himself to be the victim of circumstances. 
He is made to suffer either for wrongs he has committed 
or for the envy others feel toward him. He has, as a rule, 
been morose and exclusive. He feels that he is being ob- 
served by others ; that every one notices a peculiarity in 
him ; that others can read and control his thoughts ; that 
the newspapers direct their flings at him ; when they speak 
of rascals, of thieves, they mean him. Before long he hears 
the voices of his enemies who are trying to ferret out his 
actions ; he stops up the key-holes and draws the blinds of 
his windows. If his neighbors cannot get rid of him as 
speedily as they wish, they put poison in his food, which 
he refuses to take. He supposes himself the victim of the 
police, of socialists, of a religious sect, who will endeavor 
to influence him by electricity, through the telephone, by 
hypnotizing him, or by forcing him to inhale all sorts of 
noxious vapors. 

Paranoia with delusions of grandeur includes all those 
who imagine themselves destined to fulfil some special mis- 
sion ; the political reformers, the Guiteaus, the religious fa- 
natics, the presidents, the emperors and kings, the Goulds 
and Vanderbilts among the insane, belong to this class 
chiefly. Guiteau, descended from a father who believed 
in Mesmerism and in free-love, was particularly fond of 
reading on religious subjects ; he masturbated at an early 
age and entered the Oneida Community at the age of nine- 
teen ; when twenty-four years old he writes to his father 
saying that he was employed by Jesus Christ & Co. Some 
paranoiacs exhibit signs of the insane neurosis at a still 
earlier day. 

The children who are exclusive, who never care to play 
with others, who pray when their comrades frolic about 
— these are the very ones who develop paranoia or the 
paranoid form of dementia prascox. Moody, queer, and 
"cranky," they go along well enough until they have to com- 
pete with others in the struggle for existence, or until they 
are overcome by some severe grief, by strong emotion, by 
political or religious excitement (the election campaigns, the 



536 THE NERVOUS DISEASES OF CHILDREN. 

revival meetings, and the like) ; and then delusions, which 
may have been latent for a long time come to the fore- 
ground. The paranoiac comes of neurotic stock, in which 
insanity, hysteria, epilepsy, and chronic alcoholism have 
been common occurrences. 

Not all children who are morose and exclusive turn into 
paranoiacs, but it is well to look with suspicion and fear 
upon youthful prodigies, who discuss philosophy, or work 
at visionary schemes while other boys are engaged in sport. 
Children, as well as adults, should exhibit a modicum of 
learning and of virtues, and possibly a few vices.* 

Emminghaus describes a form of acute paranoia with 
hallucinations which is said to occur in children after febrile 
attacks, but the cases he quotes are not very convincing ; 
they are to be distinguished from ordinary mania by the 
evidence of hallucinations and by their mode of onset. 

The course of paranoia is eminently chronic ; for a time 
slight remissions may occur, enabling the person to return 
to some regular occupation. He may be able to keep his 
delusions in abeyance for a varying period of time, but they 
will come to the front in the end. The chronic stage is 
established sooner or later, in which he is entirely con- 
trolled by his delusions, and is a fit subject for asylum treat- 
ment. Very little can be attempted in this disease in the 
way of treatment, but if the first traces of the disorder are 
observed in a young boy, a special endeavor should be 
made to provide him with healthful surroundings, to di- 
vert his mind from his own person, and to arouse his 
interest in those things which are the reverse of those he 
is accustomed to brood over. Special teachers, and tours 
to foreign lands may accomplish something; but disap- 
pointment to parent, teacher, and physician is the most 
common result. 

Moral Insanity. — We need say little of this condition, 
Avhich has given rise to so much discussion. It is generally 
conceded that a lack of the moral sense may be the chief 
feature, but this moral imbecility is generally associated 
with a defect in the intellectual sphere ; hence many of the 

* In this chapter the author has drawn occasionally upon the descriptions given by 
him in his article on Insanity and Crime, in Hamilton's System of Legal Medicine. 






INSANITY. 537 

subjects of moral insanity are idiotic or feeble-minded per- 
sons. 

Maudsley, some years ago, went out of his way to defend the rights of 
moral insanity. " It maybe witnessed even in young children, who, long be- 
fore they have known what vice meant, have evinced an entire absence of 
moral feeling, with the active display of all sorts of immoral tendencies, a 
genuine moral imbecility or insanity." But the author is compelled to add 
that " associated with this defect there is frequently more or less intellectual 
deficiency, but not always ; it sometimes happens that there is a remarkably 
acute intellect with no trace of moral feeling." 

There are some, children and adults, whose intellect- 
ual faculties are on a far higher plane than their moral 
qualities, and in whom the latter cannot possibly be fostered. 
In a family, one of five or six children may be the only one 
to resist the influences of religious and secular training. 
Many authors are firmly convinced that the defect in mo- 
rality is to be ascribed primarily to an intellectual defect ; 
opposing views are held by some of the ablest writers, in- 
cluding Lombroso, Maudsley, and Hack-Tuke. Krafft- 
Ebing urges the justice of retaining moral insanity as a 
clinical form, and to this there can be no objections. 

Some years ago I saw a young man at a clinic who had been arrested for 
assault upon his mother, whom he had failed to kill. He was entirely indif- 
ferent to the charge brought against him, and when asked whether he 
thought it was proper to kill one's mother, answered, " You might as well 
kill your mother as anyone else." The young man had received no intellect- 
ual or moral training, no religious instruction, had grown up among the 
most degenerate classes, and had never received the most ordinary moral 
teachings. Naturally the moral sense was deficient. Such a condition is 
embraced in Mendel's definition of moral insanity as that form which is either 
congenital or acquired in the earlier years of life, and is characterized by im- 
becility associated with a morbid tendency to immoral actions. Binswanger 
holds very correctly that a number of mental diseases lead to" moral idiocy." 

The prognosis of the condition of moral insanity is abso- 
lutely unfavorable ; nothing can be accomplished except by 
placing such children with moral obliquities under the stern 
discipline of a reformatory or an asylum. 

Epileptic Insanity." — Mental derangement associated 

* Hysterical Insanity has been alluded to in the chapter on Hysteria (pp. 86-88). 
The author was at first inclined to discuss the entire disease in this, division on mental 



538 THE NERVOUS DISEASES OF CHILDREN. 

with epilepsy has been alluded to in a previous chapter. 
(See page 67.) It is only necessary in this connection to re- 
call the fact that a maniacal attack may take the place of an 
ordinary epileptic seizure, and that such an attack is charac- 
terized by unusual suddenness and violence. Any very sud- 
den development of mania in a child may be regarded as a 
symptom of possible epilepsy, even if other epileptic signs 
are entirely wanting. In addition to these " psychic equiv- 
alents " of an epileptic seizure, epileptic children exhibit a 
marked tendency to idiocy and dementia. I have known 
of the condition of double consciousness in an epileptic 
aged eighteen, but I have not seen it in younger subjects. 
The more pronounced symptoms of epileptic insanity are 
encountered in persons past the age of puberty, but Wilder- 
muth insists that only about twenty per cent, of infantile 
epileptics exhibit anything like a normal mental condition. 

A general psychic degeneration is a most marked feature of epileptic sub- 
jects. The disease is very common among criminals, as are other degenerative 
neuroses. Alcoholism in the parent is a powerful etiological factor. (De- 
jerine states that in 37.7 per cent, of three hundred and fifty epileptics the 
father was a drunkard.) Moreover, the epileptic boy is unable to attend 
school and falls an easy victim to bad associates. 

Paretic Dementia, the serious psychosis of middle life, characterized by a 
progressive dementia, delusions of grandeur, and a long series of physical 
symptoms (inequality and immobility of the pupils, disturbances of speech, 
tremor of the face and hands), is so rare in childhood that we do not feel 
warranted in including it among the forms of insanity occurring in childhood. 
The youngest paretic I have seen was a man, aged nineteen years. Spitzka 
observed one at eighteen years, Trumbull observed the disease in a boy of 
twelve years, who had an attack of hemiplegia at the age of ten years. The 
autopsy revealed the changes of paralytic dementia. The cases thus far ob- 
served in youthful subjects did not take as rapid a course as in the adult type. 

The author would make no mention of the disease, were it not for an ex- 
perience he had some years since with a«young man who in the course of a 
few weeks became exceedingly hilarious, developed the delusion that he was 
enormously wealthy, showed distinct tremor of speech and of hands, inequal- 
ity of the pupils, and became extravagant and grandiloquent. The diagnosis 
of paralytic dementia was made by competent neurologists ; the boy was sent 
to an asylum, where all his symptoms disappeared after a few weeks. He 
has been entirely well since. 

diseases, but hesitated to do so because the marked psychic forms of hysteria are not 
often observed in children. The mental changes in chorea have been referred to on 
page 115. 



INSANITY. 539 

Masturbation and Insanity. — There is not sufficient 
reason to erect a special type to be called " Masturbatic In- 
sanity," for the habit is present in many different forms of 
insanity, particularly in young subjects. But the presence 
of this etiological or complicating condition is always notice- 
able, and leaves a distinct impression upon the development 
of the various psychoses. In many cases of acute derange- 
ment in the young, in the various forms of dementia pras- 
cox, it is often an important and grave factor. The author 
has had under his observation young masturbators who at 
the ages of ten and twelve years displayed the first effects 
of masturbation, and at the ages of sixteen to twenty years 
presented the typical symptoms of hebephrenia (mental en- 
feeblement, silliness, depression, general restlessness, and 
irritability), and gradually developed a striking dementia. 
There is such a marked resemblance between various forms 
of insanity due to masturbation that one is sorely tempted to 
constitute them a special group. The following account 
is typical of many the author could 'give : At the age of 
twelve or fourteen years, if not earlier, the bo) T either in- 
stinctively or through the force of bad example begins the 
habit. For a time it has no marked effect upon him ; he 
continues in his studies, but does not get on quite as well 
as he formerly did. For this he finds all sorts of excuses. 
He is supposed to be, and often is, subject to severe head- 
aches ; is tired in the morning, late in rising, loses his 
pleasure in out-of-door sports. If detected at this stage 
and convinced of the viciousness of his habit he may make 
a determined effort to stop ; sometimes he succeeds ; more 
often he fails. From this period on, lack of concentration 
upon any mental effort, general irritability, disturbed sleep, 
loss of flesh, are the prominent symptoms. Morbid con- 
ceptions are often troublesome, and at times also lead to 
delusions of persecution. A young man under mv care 
who would gaze for hours upon a clock heard it say, " De- 
tec-tive, De-tec-tive," and imagined himself run clown bv 
them, but was even then too stupid to give any reason for 
such persecution. The patient leads an aimless, silly exist- 
ence ; will pore for hours over books without reading them ; 
does nothing of his own initiative, but if forced to walk 



540 THE NERVOUS DISEASES OF CHILDREN. 

or to take exercise of some sort does everything in a me- 
chanical fashion. If he be the son of wealthy parents, the 
effort is made to distract him by travel, but all is in vain ; 
he takes no interest in anything and simply watches for 
the opportunity to indulge in masturbation. Complete 
idiocy and dementia are the ultimate result. Reference 
has been made to boys, but girls also fall victims to the 
habit, and the symptoms they present are similar to those 
specified before. The prognosis of all forms of insanity due 
to masturbation is extremely unfavorable. I have succeeded 
in checking the habit in relatively few cases. Many patients 
continue on their evil course in spite of all precautions. It 
is, if anything, more difficult to control the habit in girls 
than in boys. If the person's reason and sense of shame 
can be appealed to the chances of curing him of the prac- 
tice are best; the previous mental calibre is an important 
factor; the successful cases I have seen have been in col- 
lege boys and other bright lads. If the habit has been ac- 
quired by imitation it can be checked more readily than if 
it is the result of an innate instinct, and the sign of a degen- 
erated mental state. Extreme watchfulness and the liberal 
use of the actual cautery to the spine and even to the geni- 
tals are the only possible means of effecting a cure. 

The prognosis of insanity in children has been discussed 
with reference to the several types. It is well to emphasize 
the fact once more that mental derangement in childhood 
is recovered from more frequently than is the case with the 
insanity of later years. 

A word in addition to what has been said on the treat- 
ment of the various types of insanity. The doctrines of 
heredity and of degeneration are taking a firm hold of the 
medical mind. Psychologically they may be true enough, 
but no one has yet proved that morbid hereditary influences 
cannot be overcome or counteracted. If a child has an un- 
fortunate hereditary predisposition to disease, give it every 
possible advantage which children without any hereditary 
taint enjoy. The separation of children from hysterical, 
epileptic, or otherwise degenerate parents, is not insisted on 
often enough. 



CHAPTER XXX. 

IDIOCY AND IMBECILITY. 

By idiocy we designate a permanent and complete im- 
pairment of all the intellectual faculties. Imbecility and 
feeble - mindedness denote lesser degrees of mental defi- 
ciency. Both these terms are used to designate mental de- 
ficiency in a brain not yet fully developed, whereas the 
term " dementia " is applied to those states in which all 
the faculties are lost after they have been normally de- 
veloped. Such dementia may occur at times in relatively 
young persons. 

Idiocy is of special interest, not only because of its frequent occurrence, 
but also because it demonstrates an extreme psychic condition due, in many 
instances, to tangible changes in the brain. In this respect it is not unlike 
general paresis, and the mental conditions included under these two terms 
are practically the only two psychic diseases whose morbid substratum is 
tolerably well known at the present time. In one, the highest structures 
of the brain have become disorganized and are undergoing dissolution after 
having attained normal development, while in the other defective growth or 
involution takes place before a full development has been reached. 

In former years idiots were much neglected.; they were 
considered an unfortunate class for whom but little could 
be done, and whose actual condition was scarcely worth 
studying ; but during the past twenty-five years or more 
an entire change has taken place and it is fair to say that 
no class of patients has been more frequently the subject 
of study than those with defective cerebral development. 

The earliest impetus was given in this direction by the French alienists, 
chief among them being Esquirol ; a little latter the elder Seguin called at- 
tention to the possibilities of improvement in these patients and to the proper 
methods of training ; and within the past decade or two, the interest in the 
morbid conditions underlying idiocy has led to numerous able contributions. 



542 THE NERVOUS DISEASES OF CHILDREN. 

Among English authors the names of Crichton Browne, West, Maudsley, and 
Langdon Down, of Clouston, Ireland, and Shuttleworth, deserve special men- 
tion ; in France, Bourneville has been the most successful student of idiocy ; in 
Germany, the works of Schiile, of Krafft-Ebing, of Emminghaus, and above all, 
of Griesinger, have contributed most to the advance of our special knowledge of 
this subject, and in this country idiocy has been studied carefully by Ray, 
Seguin, Mills, Kerlin, Spitzka, Hurd, Brush, Peterson, Barr, and the author. 

Classification. — The discussion of idiocy has been not 
unlike that of insanity. Each author has felt called upon 
to subdivide the subject and to establish a rational classi- 
fication. The result has been that no two authors have en- 
tirely agreed. One has attempted to give a classification 
based upon the etiology of the condition, another upon the 
pathology. Down has attempted a division based upon 
ethnic standards, the Mongolian, the Malay, the Indian, and 
the Ethiopian types. 

For purposes of convenience we will divide the subject 
as follows : 

I. Hereditary Idiocy. J < a > Congenital. 

( (b) Developmental. 

( After brain or skull injuries (including birth idiocies). 
II. Acquired Idiocy.. ■< After convulsions. 

( After infectious diseases. 
III. Myxedematous Idiocy. 

I. (a). Hereditary Congenital Idiocy includes those condi- 
tions in which the brain at birth is deficient, although 
this defect need not be apparent for some weeks or even 
months after birth. In the chapter upon Arrested Cerebral 
Development I have enumerated some of the anatomical 
conditions with which idiocy is associated, and to this part 
of the subject we shall recur later on. These congenital de- 
ficiencies may be the result of disease during the intra- 
uterine period. 

Congenital idiocy is a common occurrence in families 
with marked neurotic taint. Parents who suffer from some 
form of insanity, from hysteria, epilepsy, or chorea are apt 
to engender idiots. Among other predisposing conditions 
alcoholism and syphilitic infection of either parent are by 
far the most frequent. The importance of alcoholism in 



IDIOCY AND IMBECILITY. 543 

particular cannot be overrated. The alcoholic habits of 
the father at the time of procreation are surely a potent fac- 
tor. Syphilis of the parent must be taken into account. 
Premature delivery is not uncommon in syphilitic cases, 
and an imperfectly developed brain at the time of birth 
may be the cause of idiocy. 

Intermarriage is supposed by many to be a direct 
cause of idiocy in children, and by others the influence of 
such marriages is absolutely denied. The truth lies mid- 
way. In families without a hereditary taint intermarriages 
may be quite harmless, but if there is any, and even the 
slightest taint of insanity, or of any other serious nervous 
affection, that taint becomes intensified by such an union ; 
and since few families are entirely free from every such 
tendency the rule holds good that in the majority of cases 
intermarriages are harmful. 

Traumatism during pregnancy is another factor in the 
development of idiocy in the child. As the influences 
brought to bear are prenatal in origin, these cases may be 
included under the heading of hereditary idiocy ; and there 
is all the more reason to do this as such injuries to the 
mother are more likely to be harmful to the normal devel- 
opment of a child's brain in families with neurotic taint than 
in families whose history is entirely negative. 

I. (b) Developmental Idiocy includes that class of cases in 
which the idiocy becomes apparent at certain well-marked 
periods of life — during dentition, for instance, and at pu- 
berty. 

II. Acquired Idiocy. — This class includes a very large 
number of conditions, due to the most varying accidents 
and diseases. First and foremost, although the cause is 
operative at the time of birth, we must include that large 
number of idiocies due to traumatism during labor. The 
same conditions which give rise to cerebral birth palsies 
(prolonged labor, instrumental delivery) also give rise to 
birth idiocy. Other things being equal the application of 
instruments is not nearly as harmful as excessively pro- 
longed labor. In the histories of children who have be- 
come idiotic, it is often stated that the child was asphyxi- 
ated at birth ; that it had frequent spasms during the earlier 



544 THE NERVOUS DISEASES OF CHILDREN. 

weeks of life, and that it exhibited marked rigidities and 
palsies of the extremities. 

According to Mitchell, in 57 of 494 cases of idiocy labor lasted for more 
than thirty-six hours ; 4 of them were born with unusual haste ; in 22 cases 
forceps were applied, and 9 of these showed the impressions of the for- 
ceps on the head after birth ; 4 of them were born by version ; in 6 cases 
there was breech presentation ; 1 1 were twins, and 9 were born prematurely ; 
29 were born asphyxiated and supposed to be dead ; 89 were the last children 
of their respective mothers. 

Birth idiocy is allied to another form due to injuries to 
the head later in life ; a fall from a bench, from a chair, 
from a cradle, is often sufficient to cause serious disturbance, 
more particularly in those children who are predisposed 
by inheritance to mental disease. It is possible that the 
easier rupture of the blood-vessels in those who have in- 
herited a syphilitic or alcoholic taint may account for the 
fact that while some children escape injury after severe 
falls others are seriously affected by relatively slight acci- 
dents. 

Among the causes of acquired idiocy none is more im- 
portant to my mind than convulsions. The entire devel- 
opment of a child may be normal until a convulsion occurs, 
whether as a result of some intestinal derangement or as 
the precursor of an acute infectious disease. From this 
time onward mental decadence sets in, and a child that 
was previously healthy and of normal mental development 
begins to exhibit more and more marked mental defects 
until it reaches the condition of complete idiocy, in which it 
may remain for many years. There is no difficulty in ex- 
plaining this occurrence, for the tremendous venous stasis 
that occurs during the acme of a convulsion is sufficient to 
cause rupture of the pial blood-vessels, and a very consider- 
able hemorrhage may follow ; meningo-encephalitis, termi- 
nating in a general sclerosis^ is the natural result and the 
direct cause of the mental defect. The acute infectious 
diseases are often responsible for the development of idiocy. 
In what manner this cerebral change is brought about is 
not easy to state, but I believe that the cases thus produced 
are relatively few, and that of these some are due to an in- 



IDIOCY AND IMBECILITY. 545 

fectious encephalitis, and others to the convulsions accom- 
panying these acute infectious diseases. 

Idiocy is developed not infrequently after acute menin- 
gitis early in childhood. Idiocy, blindness, deaf-mutism — 
all these conditions separately, and sometimes conjointly, 
are the unfortunate results of early meningeal disease. The 
explanation of this condition is relatively simple, for the 
meningitis and the meningo-encephalitis, if they last long 



Hfifi&gBBiijSB^^B 


; \ 



Fig. 135. — Hydrocephalic Idiot. (Peterson.) 

enough, may lead to a general sclerosis and atrophy of the 
cortical substance, which will, prevent the further normal 
development of the brain. 

The association of idiocy with hydrocephalus is com- 
mon, but as this condition is generally a secondary state, 
both the idiocy and the hydrocephalus are the result of the 
primary disease. (See Fig. I35-) With the gradual increase 
of the hydrocephalic fluid, however, the cortical function 
naturally becomes impaired ; there are therefore few chil- 
dren with unusually large heads whose mental condition is 
at all normal, though it is quite remarkable to what extent 
the hydrocephalus may increase before an absolute aboli- 



546 THE NERVOUS DISEASES OF CHILDREN. 

tion of function takes place. This is true not only of the 
general mental condition, but also of the special sensory 
functions of the cortex. 

Idiocy and epilepsy are intimately associated with one 
another. In all cases in which epilepsy has been developed 
early in life, or in which the epileptic seizures are frequent, 
there is a natural tendency to mental deterioration. In the 
adult we speak of epileptic dementia, in younger persons of 
epileptic idiocy. There are, no doubt, gross changes in the 
brains of such subjects, and in many the seeds of both the 
epilepsy and the idiocy may be traced to injury either at 
birth or during the first years of life. Thus not only the 
idiocy but the epilepsy, as well as the palsy, may be the re- 
sult of meningeal hemorrhage occurring during labor or in 
very early childhood. 

Symptoms. — The chief characteristic of idiocy is the 
lack of ordinary mental conceptions. The brain is not able 
to receive impressions from the outer world, and if such 
are received, it is not able to utilize them in anything 
like normal fashion, nor to form concepts or judgments. 
In some instances the brain represents an entire blank, in 
others a few impressions have been received, and these 
have been developed into imperfect concepts. This abso- 
lute mental deficiency is present in typical idiots, but there 
are varying degrees of mental deficiency or of mental devel- 
opment in imbeciles or feeble-minded persons. 

It is scarcely necessary to cite cases in order to establish 
the clinical features of complete idiocy, for they are too well 
known even to laymen to need elucidation. 

If idiocy is complete the child or the adult may not be 
able to recognize its own parent. He fails to recognize any 
object or the use of such, and of course is not able to under- 
stand or appreciate what is said to him. In extreme in- 
stances the idiot is practically nothing more than a highly 
organized vegetating organism, truly animal-like in all his 
actions without the slightest trace of human intellect. 
Through faulty development of speech the defect in the 
general mental condition of the child first becomes notice- 
able. If there is a lesser degree of idiocy, the child may 
master a few concepts, may understand a few uttered 



IDIOCY AND IMBECILITY. S47 

sounds, or may be able to pronounce such simple words 
as mamma, papa, and the like. He may recognize the 
use of objects, may be capable of slight training and in- 
struction, so that the ideas of cleanliness, and of mine and 
thine may be impressed upon his mind. 

From these rather marked forms of idiocy and mental 
imbecility there is every possible gradation until we reach 
those types in which speech is only a little deficient, but 
the mental horizon is evidently narrowed down so that rel- 
atively few concepts are formed, abstract ideas are almost 
entirely wanting, and the imperfect mental status is deter- 
mined by the lack of ordinary motives for action, by the 
awkwardness in intercourse with other people, and above 
all by the inability of the individual to cope with others in 
the struggle for existence. The inferiority of the individ- 
ual is discovered not in the family circle in which all con- 
ditions are favorable, but when the child is thrown into 
competition with others at school. If the boy or young 
man is compelled to seek a living for himself, the feeble- 
minded youth is pushed to the wall under such condi- 
tions. That such persons, moreover, cannot appreciate the 
reason for their failure to make a success of life goes with- 
out saying. That they are rarely capable of developing 
the higher religious and moral ideas is equally true. The 
criminal classes are recruited largely from the category of 
feeble-minded persons in whom the ordinary ideas of right 
and wrong have not been engendered by early training. 

The general deficiency in intellect is supposed to be offset occasionally by 
a peculiar development of the mind in some one direction. Stories to this 
effect have been common, and have been repeated by one author after the 
other ; Griesinger's case has become rather famous of an idiot who was said 
to have constructed a perfect model of a man-of-war without having had 
any conception of geometrical designs. Many idiots are said to possess spe- 
cial talent in the use of cards. Drobisch described an idiotic boy. who after 
reading over a single page was able to repeat word for word, even if it was 
in Latin, which he did not at all understand. I have had under observation a 
young imbecile, a boy aged fourteen, who takes special delight in reading and 
remembering each sign that he passes on the streets, and he can without 
difficulty repeat fully two hundred names in the order in which they occur 
on the avenues. He has a true passion for signs, and as he passes along t he- 
streets his attention is riveted upon nothing else. Some of the most astound- 



548 THE NERVOUS DISEASES OF CHILDREN. 

ing lightning calculators have been weak-minded, if not imbecile, in everything 
else excepting memory of figures. It is not fair, however, to turn this about 
and to claim that every lightning calculator must necessarily be an imbecile, 
for there is no doubt that some normal minds can, by special training, reach 
an inordinate development in some one particular direction. 

As we are interested chiefly in the mental condition of 
children, we must devote a little more time to the early rec- 
ognition of idiocy. In cases of very marked mental defi- 
ciency the disordered condition of mind can be discovered 
in the first months of life. In fact if parents and physicians 
had a proper understanding of such conditions, the mental 
defect could be discovered very much earlier than it gen- 
erally is. It is most instructive in this connection to con- 
sider the observations of Preyer, who, in his famous treatise 
on the psychic development of the child, shows how early 
the mental processes of a healthy child can be discerned. 
It is a surprise to most of us to learn from him that a child 
in the second day of life was able to distinguish between 
light and darkness ; that at a very early day it appreciates 
the sounds of words, and that in its cooing utterances it 
exhibits evidence of considerable cerebration. Very few 
people will, of course, observe children as Preyer observed 
his own child, but mental deficiency can, as a rule, be made 
out if the parent or the physician take the trouble to com- 
pare the actions of a child with the average healthy child 
at the same age. It is important, therefore, to note the time 
of life at which children may be expected to exhibit certain 
signs of intelligence. (See page 7.) If children have not 
acquired speech at the end of two and a half years, and par- 
ticularly if they fail to understand what is spoken, it is fair 
to infer that there will be a distinct mental defect later on. 

Wildermuth has taken the trouble to tabulate a number of the more im- 
portant stigmata of degeneration commonly found in idiots. Eighty-two per 
cent, of all idiots according to these statistics present some such signs. There 
were abnormal conditions of the fundus in 6 of 142 cases ; malformations of 
the external ear in 53 ; abnormal position of the teeth in 32 ; flattening of 
the hard palate in 1 1 ; highly arched palate in 30 ; prognathism in 9 ; ex- 
cessive thickening of the skin in 9 ; asymmetry of the face in 25 ; and abnor- 
malities of the genital organs in 8 cases. Among the functional stigmata 
Wildermuth refers to anomalies of the tendon reflexes in sixty per cent. ; 
inco-ordination of the lower extremities in ten per cent. ; and in six per cent. 



IDIOCY AND IMBECILITY. 



549 



squinting occurred. It is a curious fact that these signs of degeneration are 
present not only in the truly congenital forms of idiocy, but also in those 
which have been acquired some time after birth. 

The mere appearance of the child or of the youth will 
often be sufficient to lead to the suspicion of idiocy without 
an examination of the 
mental condition itself. 
The entire absence of 
speech, or of defective 
speech in many cases, will 
point the same way. Cau- 
tion should, however, be 
exercised in not mistaking 
deaf -mutism for idiocy, 
and if a child cannot 
speak, careful examina- 
tion should be made in 
other ways to determine 
its intelligence ; not rare- 
ly, however, deaf-mutism 
and idiocy are combined, 
and at times the former 
has been the direct or in- 
direct cause of the latter. 

Among idiots the de- 
fects of skull formation 
are of especial interest. 
The microcephalic skull 
is particularly frequent, 
and is either small in all 
its dimensions or ample 
in some and very defi- 
cient in others. (See Fig. 
136.) The transverse and 

occipital diameters may be entirely normal, indeed the 
horizontal circumference may be up to the average, and 
yet if some such heads are examined it will be found that 
the frontal portion is unusually small, possibly receding, 
while the middle and occipital portions may be entirely 
normal. Such discrepancies and such asymmetry should 




Fig. 136. — Congenital Idiot ; Microcephalic 
Skull ; Extreme Contractures of Adductor 
Muscles of Thighs. (From a photograph 
kindly furnished by Dr. Peterson.) 



550 THE NERVOUS DISEASES OF CHILDREN. 

be carefully noted, for it is more important than slight 
deficiencies in the total measurements. Smallness of the 
anterior half of the skull with receding forehead proves 
either that there is very little room for the brain, or that 
the brain being small requires very little space. Asymmetry 
of the skull is particularly frequent, as has been shown by 
Fisher and Peterson, in those cases in which idiocy is asso- 
ciated with early infantile hemiplegia. Another point to be 
remembered is that irregularities in the structure of the 
skull are not uncommon in healthy individuals, and that as 
long as the cubic contents of the cavity of the skull are 
near the average, defects of one kind or the other appear to 
play a very small part. In various parts of this book I 
have referred to my belief that the growth of the skull is 
dependent largely upon the condition of the brain within, 
and this accounts for the fact that in idiocy acquired after 
convulsions the growth of the skull ceases with the dis- 
turbed condition of the brain ; microcephalus is found 
among acquired idiots as well as among congenital idiots. 

The general restlessness of idiots is characteristic. In 
my lecture-room I allowed these children full sway in order 
to demonstrate this special feature to my class. They are 
about the only patients who concern themselves little about 
the presence of the students, and who roam about the entire 
room in an uneasy fashion, taking hold of everything, pul- 
ling down what they can, and always fearless of the conse- 
quences. If they cannot employ themselves in any other 
way, they will keep up a constant motion with the fingers, 
twisting and braiding them, allowing the nails to scrape the 
skin off the fingers. They bite their nails and are apt to 
tear their garments. There is frequently dribbling and 
drooling, and if opportunity is offered, these children are 
particularly fond of putting their tongues to the window- 
panes, or any other cold object. The general awkward- 
ness of the movements, excitability and irritability of tem- 
per, together with a peculiarity of carriage and blankness 
of facial expression, complete the clinical picture of idiocy 
and imbecility. 

Imbeciles of lesser degree often escape detection ; some of them are suffi- 
ciently conscious of their defects to conceal them in the presence of others. 



IDIOCY AND IMBECILITY. 551 

As they grow older the deficiency in the mental and moral make-up is evi- 
denced in the excessive development of the animal appetites. Masturbation 
is developed in early years, the effect of which adds to the mental deteriora- 
tion. If the sexual appetite is thoroughly aroused, gratification is sought in 
the most outrageous fashion, and intercourse may be attempted with children 
or old women. Giraud referred to an idiot who attempted to rape his own 
sister. All the ordinary feelings of shame and modesty are wanting. In some 
instances the defect is a little more marked in the emotional or moral spheres 
than in the intellectual. Such a condition has generally been termed one of 
moral imbecility ; but it is always associated with a decided intellectual defect. 
Weak-minded and imbecile children commit crimes either from defective 
judgment as to the consequences or from the simple desire to gratify their 
passions and impulses. The desire to witness a grand spectacle or to 
revenge himself upon others, has led many an imbecile to set fire to houses, 
regardless of all consequences. 

Pathology. — We have stated that idiocy is rarely a 
primary condition, that it is frequently associated with 
other conditions which point to serious brain trouble. A 
complete account of the pathology of idiocy would include 
the terminal stage of very many different brain diseases. 
While it is interesting to note these various terminal states 
it helps us but little in determining the primary lesion. 
Thus Wilmarth has described one hundred brains of idiots. 
Among these were found conditions of sclerosis with atro- 
phy, of tuberous sclerosis, of general diffuse sclerosis. He 
described, furthermore, brains in which the most marked 
features were degenerative changes in the vessels and higher 
nerve-cells. He also mentioned hydrocephalus, general atro- 
phy, and the like, so that it would be difficult from this 
enumeration to make any sort of inference as to the primary 
morbid state. The attempt should be made to distinguish 
between the primary and terminal states, and thus help us 
to push on, however little, toward a final solution of the 
question. I believe we shall do best if we accept the clini- 
cal division of idiocy adopted in this chapter, and endeavor 
to arrange the known morbid processes as far as possible 
under these larger clinical subdivisions. 
41 



552 



THE NERVOUS DISEASES OF CHILDREN. 



Hereditary idiocy. 



Acquired idiocy. 
(a) Birth palsies. 



(b) After acute dis- 
eases and convul- 
sions. 

(c) From trauma- 
tism. 



Primary Lesions. 



Large defects ; hemicephalus 
or entire absence of a con- 
siderable portion of a hemi- 
sphere. 

Partial defects (porenceph- 
aly) ; most frequently in 
motor regions. Single or 
double. 

Small brain (microcephalus) ; 
all parts equally developed, 
or arrest of development 
more marked in frontal and 
occipital portions than in 
other parts. 

Agenesis corticalis ; brain ap- 
parently normal or showing 
only slight changes in ex- 
ternal configuration ; mal- 
development and disintegra- 
tion of cells and fibres of 
cortex. 

Intra-uterine, inflammatory, or 
vascular disturbances, such 
as encephalitis, thrombosis, 
and hemorrhage. 

Meningeal hemorrhage. 



Meningitis, encephalitis (sim- 
ple or hemorrhagic) ; throm- 
bosis (specific endarteritis). 

Meningeal hemorrhage ; en- 
cephalitis. 



Terminal States. 



Same ; often compensatory de- 
velopment of hydrocephalus 
with large cysts. 

Same ; with addition of hydro- 
cephalus, local cysts, and gen- 
eral sclerosis with atrophy. 

Small brain, often hard and 
sclerotic, sometimes compen- 
satory hydrocephalus. 



Same ; no sign of inflamma- 
tory conditions, blood-vessels 
scanty ; some external hydro- 
cephalus. 



Meningo - encephalitis ; sclero- 
sis, localized or diffuse ; gen- 
eral atrophy ; cysts. 



Meningo - encephalitis, local 
cysts, sclerosis, and general 
atrophy. 

Meningo -encephalitis, sclero- 
sis, diffuse atrophy. 

Meningo-encephalitis, abscess, 
sclerosis, and atrophy. 



That there are other conditions which occasionally lead 
to idiocy cannot be doubted, but they are, I am sure, not 
nearly as important nor as frequent as those mentioned in 
the above table. If hereditary syphilis is the etiological 
factor it is more than likely to give rise to idiocy through 
the medium of local meningitis, encephalitis, or of throm- 
bosis with it's subsequent changes. Mills insists on idiocy 
of toxic origin, and includes under this term cases due to 
acute poisoning, or those following acute infectious dis- 
eases, such as measles, scarlet fever, and the like. 

Diagnosis. — Idiocy is recognized easily enough, except 
in very young children ; a comparison with the attainments 
of other children of the same age will help to establish the 
fact of an inferior mental development. If a child presents, 
in addition to the mental symptoms, any of the physical 
stigmata of degeneration, the probability of its remaining 



IDIOCY AXD IMBECILITY. 553 

an idiot is very great. Defective development of speech 
after a child has passed the third year is also an important 
aid in diagnosis, if this condition is not the result of deaf- 
mutism. 

The Prognosis in the majority of cases of idiocy is bad. 
If a child has not learned to speak, and has not acquired 
the simplest concepts at the age of three or four years it will 
remain backward for all time. In the case of imbeciles of 
lesser degree the prospect is not quite so gloomy. In this 
condition more can be expected from the careful training 
on the part of parents and teachers. 

Treatment. — The treatment of idiots is, as a rule, re- 
stricted to watching over their physical development and 
securing for them proper hygienic surroundings. In fam- 
ilies such children are a torment to their parents and a bad 
example to brothers and sisters. Their separation from 
home is advisable, and should be urged in spite of all senti- 
ment to the contrary. The patients enjoy the greater 
liberty which special institutions afford them, and the family 
is better off without them. 

Imbeciles and weak-minded children require more care- 
ful consideration. By the most diligent training at the 
hands of experienced persons much can be done for them 
to bring their behavior and their physical condition as 
closely as possible to that of the average child. I am a 
thorough believer in pedagogic methods and of such only. 
If at all possible, such children should be intrusted at a very 
early day to a competent teacher, who will take the time 
to study the peculiarities of the individual, and who will 
fasten upon and endeavor to develop the few signs of an 
intellectual awakening. It is often surprising to see how 
much can be accomplished w T ith these unfortunates. In 
some instances, however, the task is a hopeless one, and all 
the evidences of imbecility can rarely be eradicated. By 
careful calisthenic exercises, and proper gymnastic training, 
much can be done to avoid the outward appearance of im- 
becility to which parents so seriously object. If special at- 
tention is paid to the development of speech, defects in this 
direction can be corrected. The larger institutions, such as 
the Bicetre in Paris, and the various institutions for the 



554 THE NERVOUS DISEASES OF CHILDREN. 

feeble-minded in this country, attain a fair measure of suc- 
cess. But, after all, only those who possess a modicum of 
intellectual development can be benefited by these meth- 
ods, whereas the severer grades of imbeciles and of idiots 
cannot be improved by them. 

Cranial surgery has been looked to as a final resort. If 
the imbecility or the idiocy is distinctly due to a small skull, 
and this small skull is the result of a premature synostosis 
of the sutures, the surgical procedures, as practised by Lan- 
nelongue, Keen, Gerster, and others, may be allowable ; but, 
after all that can be said on this subject has been heard, 
there is but little reason to expect much improvement. 
The slight changes that have been noticed and have been 
reported in children after craniectomy scarcely warrant 
the procedure, and, as has been intimated in a previous sec- 
tion, the dangers of the operation are so great that crani- 
ectomy should only be practised at the urgent request of 
the parents, and after every other method has been given a 
fair trial and has failed. In those patients in whom there is 
evidence of a defective cerebral development, independently 
of the condition of the skull, any operation is, on a priori 
grounds, entirely useless. 

Medication by drugs is rarely beneficial ; yet some phy- 
sicians will undoubtedly be impelled by the evidence of an 
existing anaemia, of rickets, or of hereditary syphilis, to ex- 
hibit the drugs commonly used in these conditions. 

III. MYXEDEMATOUS IDIOCY — SPORADIC CRETINISM. 

This condition, which was well described by Bourneville many years ago, 
deserves special mention. The mental condition is a very striking feature of 
these children, but it is simply a part of the general stunting of the physical 
and mental growth. The condition itself, although a very rare one, is of 
more interest at the present day in connection with the satisfactory studies 
that have been made regarding the cause and the treatment of myxcedema. I 
have associated myxcedematous idiocy with sporadic cretinism, because the 
two seemed to me to be very closely related, the cretin representing, how- 
ever, a more intense development of the affection than is met with in those 
cases described as myxcedematous idiocy by Bourneville. Cases of sporadic 
cretinism in every way resembling the endemic cretinoid cases, so common 
in the mountainous districts of Europe, occur also in a few American re- 
gions, as in the mountains of Vermont and California. The cases which we 



IDIOCY AND IMBECILITY. 



555 



have the opportunity to see in the larger American cities are from the for- 
eign element of the population, and though we speak of them as cases of 
sporadic cretinism, they occur generally in descendants of families who have 
lived in regions in which cretinism has been endemic. 

The child, whose picture is reproduced in this chapter, is a typical case of 
this kind. The boy was born of healthy Prussian parents, who immigrated 
to this country some seven or eight years ago. He has always been healthy, 
but ever since he was a year and 
a half of age, the parents have 
been struck by the fact that 
he did not seem to continue 
growing as the other children 
in the family did. This stunt- 
ed physical growth was asso- 
ciated with an impairment in 
the development of speech and 
of all other faculties. 

The child might not unnat- 
urally, at the age of twelve, be 
taken to be three, or not much 
more than three years old. The 
boy has learned to speak a few 
words, is able to call his par- 
ents and some near relatives. 
His affections are well devel- 
oped, as was evidenced when 
he was separated from his father 
and placed in the hospital, and 
he shows considerable aptitude 
in playing with other children, 
but not quite as much as a 
healthy child of three years of 
age would. As for his mental 
condition, it is not equal to that 
of a child of three years; be- 
yond the expression of his sim- 
ple wants, he exhibits remarka- 
bly few signs of mentality. 

The disease is characterized 
by a peculiar glossy appearance of the skin, prominent lips, receding fore- 
head, and a peculiar, stubbed nose. Compared with the rest of the body the 
Stomach is inordinately large ; the tongue is relatively thick, and very often 
protrudes from the mouth. All children with myxcedematous disease so 
closely resemble each other that they might be supposed to belong to one and 
the same family. The examination of the neck fails to reveal any trace of 
the thyroid gland. 

Etiology. — The etiology of myxcedematous idiocy, as well as of spora- 




Fig. 137.— Case of Myxoedema with Idiocy. Pa- 
tient Twelve Years Old ; Dwarfish in Stature. 



556 THE NERVOUS DISEASES OF CHILDREN. 

die cretinism, is practically unknown, except that it is very apt to occur in 
mountainous regions, and particularly in regions in which the water contains 
a great deal of salts of lime and magnesia. But this is evidently not the 
only explanation, for the children of persons who have emigrated from such 
districts have often been affected with the disease although subject to en- 
tirely different atmospheric and tellurian conditions. The frequent intermar- 
riages between people in isolated mountainous districts also has its bearing 
upon the hereditary transmission of these diseases.* 

Diagnosis. — Myxcedematous idiocy cannot very well be mistaken for 
any other condition. It is only in the earlier years of life, when the stunted 
growth and the peculiar expression of the child are not yet fully marked, that 
the true cause of the idiocy may not be suspected. But the arrest in the 
general growth and the peculiar appearance of the skin will give the clew to 
the true state of affairs.f 

Prognosis. — The prognosis of this condition was extremely grave until 
very recently, and therapeutic experiments are now proceeding which will 
prove whether this one class of idiots may possibly be benefited by treatment. 
Until a decade or more ago there was as little hope of improving myxcedematous 
patients as of helping other congenital idiots, and a removal from the moun- 
tainous region in which cretinism was endemic, or an entire change of sur- 
roundings, was supposed to be the only possible way of procuring some relief, 
though actual cure of any such condition had not, to my knowledge, been 
recorded by anyone. » 

As will be seen in the discussion of the treatment of this condition there 
is some hope of improving these children by the use of recent methods. 

Treatment. — All the older suggestions with regard to treatment can 
be discarded. Feeding the patient with the thyroid gland promises some re- 
lief. The author has been personally interested in the treatment of several 
patients with myxcedematous idiocy. In one of them a very decided change 
was effected, the mental and physical symptoms being greatly benefited by 
the' use of thyroid extract. In two other cases the treatment was entirely 
unsuccessful, and in a fourth patient, a cretin, aged thirty-four years, a con- 
siderable change in the physical condition was effected, but the patient 
became so emaciated that the drug had to be discontinued.! The pulverized 
gland is now for sale in the market and can be obtained easily in any quan- 
tity. With children the treatment should be begun with doses of one grain 
of the thyroid gland, to be given three times a day, and increased from one 

* For the pathology of this condition, see page 199. 

t These conditions bear a superficial resemblance to infantilism — a condition char- 
acterized by stunted growth, a persistence of child-like physique, non-development of 
the genital organs, and of those physical and mental traits which go with normal sexual 
growth (Bourneville, Brissaud, Meige, Lorain, and other French and Italian writers). 
There is some resemblance also to achondroplasia — a stunted growth of the extremities 
with normal growth of the trunk (Marie). Skull relatively large. The epiphyses are 
deformed and thickened. Intelligence, as a rule, remains normal. 

X All these cases were treated more than ten years ago. With the improved 
method of administering the " gland," better results have been obtained. 



IDIOCY AND IMBECILITY. $$7 

up to five grains. While the drug is being given the child should be under 
proper medical observation, and the effect of the administration of the gland 
upon the heart and other organs should be carefully noted. This treatment 
should be begun in the earliest stages of myxcedematous idiocy, with the view 
to prevent, if possible, the full development of the disease. 



INDEX. 



INDEX. 



Abducens nerve, in tumor of cerebel- 
lum, 479 
Abscess of brain, 488 

chronic abscess of, 492 

differential diagnosis, 493 

prognosis, 494 

treatment, 495 

of cerebellum, 490 
Acute ascending paralysis. See Lan- 
dry's paralysis, 283 
Agenesis corticalis, 399, 513 
Agoraphobia, 524 
Amaurotic family idiocy, 462 

symptoms, 463 

etiology, 464 

diagnosis, 465 

pathological anatomy, 465 

prognosis, 468 

treatment, 469 
Amyelia, 386 

Amyotrophic lateral sclerosis with bul- 
bar symptoms, 420 
Amyotrophies and myopathies, distinc- 
tion between, 353-354 
Anaemia, headaches due to, 167 
Anaesthesia, 37 

hysterical, 98 
Aneurism, cerebral, 484 
Angio-neurotic oedema, 201 
Angio-paralytic, angio-spastic forms of 

migraine, 179 
Ankle clonus, 40 
Anosmia (loss of smell) in tumor of 

frontal lobe, 475 
Antitoxin of tetanus, 152 
Aphasia, 439 

aphasia in migraine, 177 
Aphonia, hysterical, 95 
Appendix, 561 

Aran-Duchenne type of progressive mus- 
cular atrophy, 353-355 
Arm, palsies of, 210 
Arsenic, method of administering in 

chorea, 126 
Astasia-abasia, 94 
Atelomyelia, 386 

Athetosis, 442, 445. See also "Double 
athetosis." 



Atrophy. See progressive muscular 

atrophies. 
Auditory nerve. See Hearing. 
Auditory symptoms in epilepsy, 67 
Aura, in epilepsy, 69 

Basal Ganglia, tumor of, 477 
Basedow's disease, 192 

cardinal symptoms of, 192 

theories of, 196 

treatment of, 197 
Baths, efficacy of, in chorea, 125 
Bell's paralysis, 216 
Birth palsies, 436 

morbid anatomy of, 447 
Blepharospasm, 225 
Blood-vessels, dilatation of, in the brain 

in chorea, 120 
Bouche de tapir in progressive muscular 

atrophy, 375 
Brain abscess, 488 

symptomatology of, 491 

defective development of, 501 

development of, 1 

diseases of, 391 

hypertrophy of, 514 

of tumors in the, 470 
Bromides, mode of administering in epi- 
lepsy, 81 
Brovra-Sequard's paralysis, with tumors 
of spinal cord, 323 

in syphilis of the spinal cord, 294 
Bulbar palsies, 418 

Caput obstipum, 15 

Catalepsy, 04 

Cephalic tetanus, 147 

Cerebellar type of hereditary ataxy, 344 

Cerebellum, tumor of, 47c) 

Cerebral development, arrest of, 462, 

501, 446 
Cerebral diplegia, family forms of, 400 

Cerebral neurasthenia, 524 
Cerebral palsies, 430 
Cerebral tumors, 470 

Cerebral tyj e of hereditary spastic- par- 
alysis, 345^446 
Cerebro-spinal meningitis, 404 



565 



566 



INDEX. 



Cervical segments in plexus lesions. See 

appendix, 561 
Charcot-Marie type of progressive mus- 
cular atrophy, 359 
Chorea, 108 

complications of, 116 

duration of, 117 

electrical reactions in, 115 

germ theory of, 112 

hereditary form of, 129 

hygienic care in, 125 

mental disturbance in, 115 

morbid anatomy and pathology of, 
119 

relapses in, 123 

treatment of, 124 
Chorea electrica, 136 
Chorea insaniens, 115 

of Sydenham, 108 
Choreiform diseases, 129 
Choreiform movements, after cerebral 

palsies, 442 
Circular insanity, 532 
Clonus, ankle, 40 
Color-sense, in hysteria, 100 
Complex tics, 134 
Compression of spinal cord, 315 
Congenital diplegias, 350, 439 
Congenital paramyotonia, 141 
Conjugated paralysis, 12 
Contracture in cerebral palsies, 440 
Contracture in myelitis, 275 
Contracture rheumatismal des nour- 

rices, 156 
Convulsions, 51 

causes of, 52 

diagnosis of, 62 

frequency of, 56 

in epilepsy, 68 

in hysteria, 89 

in lead-poisoning, 247 

partial, significance of, 57 

prognosis, 63 

theories of, 55 

treatment of, 64 
Convulsive tremor, 142 
Coprolalia, 225 
Cornu ammonis, disease of, as a cause 

of epilepsy, 77 
Corpora quadrigemina, tumor of, 477 
Cortical tumors, 474 
Cranial measurements, 5 
Cranial nerve nuclei, defective develop- 
ment of, 515 
Craniotabes, 60 

Craniotomy, in infantile cerebral pal- 
sies, 460 

in idiocy, 554 
Cretinism, sporadic, 554 
Crus cerebri, tumor of, 477 
Cyclops, 501 



Cysts of brain in infantile cerebral 
palsy, 450 

Defective development of brain, 501 
Deformities in infantile spinal paralysis, 

256 
Degeneration, in the brain, 

in idiocy, 548 

in insanity, 540 
Degeneration, electrical reaction, 44 
Dementia, 533 
Dementia prsecox, 533 

in hereditary chorea, 130 
Diet, in chorea, 125 
Dietary, in epilepsy, 83 
Diphtheretic paralysis, 240 

course and duration of, 242 

pathological anatomy of, 243 
Diplegia, 457 
Diplomyelia, 387 
Diplopia, 12 
Disseminated sclerosis, 304 

atypical forms of, 310 

differentiation from paralysis agitans, 

etiology of, 307 

pathology of, 308 

prognosis of, 313 

symptoms of, 304 

treatment of, 313 
Dolichocephalus, 6 
Double athetosis, 446 
Duchenne's type, 353-355 
Dystrophies, progressive muscular, 368 

Ear Disease, as a cause of headache, 
171; of abscess, 489; of sinus throm- 
bosis, 499 
Echolalia, 137 
Eclampsia infantum, 51 
Electrical conditions, table of, 44-45 

examination, 42 

examination in progressive neural 
muscular atrophy, 362 

reactions in chorea, 115 

reactions in progressive muscular 
atrophies, 355-377. 
Electricity, service of, 45 

use of, in chorea, 127 
Embolism in chorea, 121 
Encephalitis, acute, 391, 416 

hermorrhagic, acute, 416 
Encephalocele, 501 
Enuresis nocturna, 191 
Epidemic cerebro-spinal meningitis, 404 

symptoms of, 407 
Epilepsia nutans, 135 
Epilepsy, 67 

aurae in, 69 

causes of, 69-71 

diagnosis of, 74 



INDEX. 



$67 



Epilepsy, Jacksonian, 69. See Partial 
Convulsions. 

pathological anatomy of, 77 

pathology of, 79 

prognosis of, 76 

procursive, 71 

psychic symptoms of, 69-70 

surgical treatment of, 83 

symptoms of, 67 

temperature in, 71 

treatment of, 80; Flechsig's method, 
82 _ 

varieties of. 66 
Epileptic aura, treatment of, 83 
Epileptic insanity, 537 
Epileptic equivalents, 68 
Epileptiform convulsions, diagnosis from 

chorea, 119 
Erb's palsy, 210 

Erb's type of progressive muscular atro- 
phy , 374 
Essential paralysis of children, 249 
Examination, method of, 1 

scheme of, 3 
Exophthalmic goitre, 192 
Eye-strain, cause of headache, 174; re- 
lation to migraine, 181 
Eyes, conjugate deviation of, 9 

muscles of, 10 

Face, asymmetry of, 12 

motor points of, 17 
Facial hemiatrophy, 204 
Facial palsy, 216 

a form of obstetrical palsy, 215 

electrical changes in, 218-219 

treatment of, 223 
Facies myopathique in progressive mus- 
cular atrophy, 374 
Facio-scapulo-humeral type of progres- 
sive muscular atrophy, 374 
Flechsig's treatment of epilepsy, 82 
Friedreich's disease, 334 

diagnosis of, from hereditary chorea, 

131 
differential diagnosis of, 339 
pathological anatomy of, 340 
reflexes in, 337 
symptoms of, 335 

Gait, examination of, 35 

Gangrene, symmetrical, 203 

Gastric disturbances, headache due to, 

171 
Genital irritation, headaches due to, 171 
Glioma of brain, 570 
Gliosis of spinal cord, 329 
Glosso-labio-pharyngeal paralysis, 420 
Glycosuria, 203 
Graefe's symptom, 194 
Grand mal, 68 



Graves's disease, 192 
Gyrospasms, 135 
causes of, 135 

Habit chorea, 134 
Hemoglobinuria intermittent, 203 
Headaches, 166 

a symptom of brain tumor, 558 

classification of, 166 

in organic disease of the brain, 172 
Head pains, location of, 167 
Hearing, examination of, 12 
Heart, affections of, in chorea, 116 
Hebephrenia, 533 
Hemianaesthesia, 40 

hysterical, 98 
Hemianopsia, 9 

in cerebral palsies, 439 
Hemiatrophy of face, 204 
Hemichorea, 11 3-1 17 
Hemicrania, 175 

Hemiplegia, 430, 433, 441, 449, 542 
Hemorrhage, cerebral, 449 
Hereditary ataxy, 334 
Hereditary chorea, 129 

differential diagnosis from post hemi- 
plegic chorea, 131 

pathological anatomy of, 132 

symptoms of, 130 
Hereditary chorea, without dementia, 

x 33 

Hereditary diseases of spinal cord, 331 
Hereditary spastic paralysis, 345 
the cerebral type of, 345, 446 
spastic paralysis of spinal origin, 345- 

35° 
Heredity, influence of, in insanity, 520 
Heredo-ataxie cerebelleuse, 344 
Heterotopia, 386 
Huntington's chorea, 129; prognosis in, 

treatment of, 133 
Hydrencephalocele, 501 
Hydrocephalus, 420 

a cause of epilepsy, 77 

acquired internal, 420-426 

chronic, 423 

congenital, 424 

indications of, 6 

operative procedures for, 428 
Hydrorrachis externa, 387 
Hygiene, importance of, in chorea, 125 
Hypesthesia, 37 

Hyperemia, headaches due to, 170 
Hyperesthesia, 40 
Hypertrophic and progessive interstitial 

neuritis of childhood, 385 
Hypochondriasis, 526 
Hypoglossal nerve, spasm of, 326 
Hysteria, 86 

diagnosis of, 102 



568 



INDEX. 



Hysteria, duration of, 103 

irregular manifestations of, 92 

manifestations of, 87 

motor manifestations of, 89 

muscular paralysis in, 94 

sensory symptoms, 97 

pathology of, 103 
Hysteria, psychic or mental, 87 

treatment of, 104 

visceral symptoms of, 100 
Hysterical anaesthesia, transference of, 

99 
anorexia, 100 
chorea, differentiation from hereditary 

chorea, 132 
insanity, 537 
paralysis, differentiation from acute 

myelitis, 284 
Hystero-epilepsy, diagnosis of, 92 

relationship to true epilepsy, 88 
Hysterogenic zones, 99 

Idiocy, 541 

acquired, 543 

classification of, 542 

developmental, 543 

hereditary congenital, 542 

signs of degeneration in, 548 

with epilepsy, 444 

amaurotic family, 462 
Imbecility, 541 

Imperative conceptions, 139, 522 
Imperative movements, 135 
Infantile cerebral palsies, 430 

classification of, 447 

differential diagnosis, 455 

distribution of, 433 

due to embolism and thrombosis, 449- 

452 

due to hemorrhage, 449 

etiology of, 435 

form of palsy, 438 

morbid anatomy, 446 

prognosis of, 456 

treatment, 457 

with epilepsy, 444 

with idiocy, 444 
Infantile oculo-facial palsy, 516 
Infantile spinal paralysis, 249 

morbid anatomy and pathology of, 
258 

prognosis in, 267 

symptoms of, 249 

theory of, 263 

treatment of, 268 
Infectious disease, the cause of menin- 
gitis, 414 

headache due to, 173 
Inflammation of nerves, 209, 238 
Influenza, the cause of meningitis, 415 
of encephalitis, 416 



Insanity, 519 

etiology of, 520 

forms of, 522 

frequency of, 520 

treatment of, 540 
Insomnia, 186; in polyneuritis, 239 
Insufficiencies, ocular, relation to head- 
ache, migraine, 174, 181 
Intra-cranial sinuses, thrombosis of, 497 

Jacksonian (partial) epilepsy, 69 
Jaw-jerk, 40 
Jumpers, 138 

Juvenile form of progressive muscular 
atrophy, 374 

Katatonia, 533 
Knee-jerk, 40 

Landouzy-Dejerine type of progres- 
sive muscular atrophy, 374 

Landry's paralysis, differentiation from 
multiple neuritis, 237 
differentiation from acute myelitis, 283 

Lannelongue's operation, 509 

Laryngeal chorea, 114 

Laryngismus stridulus, 59 

Lead encephalopathy, 247 

Lead paralysis, 246 

Leg, palsies of, 225. See also Para- 
plegia. 

Leg type of progressive muscular atro- 
phy, 359 

Lenticular nucleus, affection of, in cho- 
rea, 122 

Leptomeningitis. See meningitis. 

Leptomeningitis, specific, 293 

Lid reflex, light reflex, 10 

Little's disease, diagnosis of, from hered- 
itary spastic paralysis, 350 

Localization in spinal cord. (See Ap- 
pendix), 561 
of tumors, 474 

Lumbar puncture, 46 

Macewen's symptom, 474 
Macrocephalus, 514 
Main en griff e, 27 
Maladie des tics convulsifs, 137 
Malaria, headaches due to, 173 
Malformations and defective develop- 
ment of the cord, 386; of the brain, 

589 
Mania, 547 

Masturbation and insanity, 539 
Megrim, 175. See Migraine. 
Melancholia, 529 

attonita, 531 
Membranes, diseases of. See Menin- 
gitis and other diseases of brain. 



IXDEX. 



569 



Membranes of the spinal cord, 314 

tumors of, 321 
Meningeal hemorrhage at birth, 448 
Meninges. See Membranes. 
Meningitis, 391 

acute, 391 

chronic basilar, 415 

epidemic, 404 

forms of, 391 

infectious diseases, 391 

and influenza, 415 

and otitis media, 412 

serosa, 421 

traumatism, 411 

treatment of, 395, 403, 410 

tubercular, 397 
Meningocele, 388, 501 
Meningo-myelrtis, specific, 293 
Meningo-myelocele, 388 
Mental disturbance in chorea, 115 
Mental excitement, control of, in chorea, 

127 
Microcephalus, 507 
Micromegaly, 387 
Migraine, 175 

coular insufficiencies in, 181 

diagnosis of, 182 

etiology of, 178 

pathology of, 179 

periodicity of, 179 

prognosis of, 182 

relation of, to epilepsy, 180 

treatment of, 183 
Moral insanity, 536 
Motor area, tumor of, 475 
Motor points, 17, 23, 25, 29, 32, ^3 
Multiple neuritis, 227 

forms of, 233 

pathological anatomy of, 2 7,^ 

symptoms of, 227 

treatment of, 238 
Multiple sclerosis, diagnosis from Fried- 
reich's disease, 340. See also dis- 
seminated sclerosis. 
Muscles of arm, forearm, and hand, 22 

of back and trunk, ^^ 

of the head and neck, 15 

of the eyes and face, 10, n 

of pelvic girdle and lower extremities, 
28, 30 

of shoulders and upper extremity, 16 

of the tongue, palate, and pharynx, 13 

total absence and early atrophy of, 384 
Muscular hypertrophy, physiological, 

377 

irritability in Thomsen's disease, 140 
movements in hereditary chorea, 130 

Muscular pseudo-hypertrophy, 371 

Muscular sense, 37 

Muscular strength, tests of, 34 

Mutism, hysterical, 95 



Mydriasis, 11 
Myelitis, acute, 272 

differential diagnosis, 282 

pathology and morbid anatomy of, 278 

specific, 293 

symptoms of acute form, 272 

treatment of, 285 
Myopathies, 268 

diagnosis, 377 

pathology, 379 

treatment, 383 
Myosis, causes of, 12 
Myotonia congenita, 139 
Myotonic reaction, 140 
Myoclonus epilepsy, 142 
Mysophobia, 523 
Myxcedema, 199 

treatment of, 201 
Myxedematous idiocy, 201, 544 

Nerves, cranial, degeneration and in- 
flammation of, 209 

facial, disease of, 216 

motor, of the eye, 10 

of arm, forearm, and hand, 22 

of back and trunk, 33 

of head and neck, 15 

of pelvic girdle and lower extremities, 
36 

of shoulders and lower extremities, 16 

of tongue, palate, and pharynx, 14 
Nerve-trunks, inflammation of, 209 
Nervous system, organic diseases of, 206 

general functional diseases, 4S 
Neurasthenia, cerebral type of, 524 

headache due to, 169 
Neuritis, 227 240 See also 385. 
Neuroglia, hyperplasia of, in epilepsy, 78 
Nictitation, 225 
Nonne-Marie type of cerebellar ataxy, 

344 
Nystagmus, 9 

Obstetrical palsies, 210 

diagnosis of, 212 

pathology of, 211 

treatment of, 214 
Occipital lobe, defective development of, 

5 11 
tumor of, 476 
(Edema, anigo-neurotic, 201 
Ophthalmoplegia, externa and interna, 
12 
partial and complete, 41S 
Opisthotonus, 143. 15S 
Optic thalamus, affection of, in chorea, 

122 
Otitic disease, relation of, to brain ab- 

->, 4S9. See Ear disease. 
Oxycephalus, 6 



570 



INDEX. 



Pain, $6 

Palsy. See Paralysis. 
Paradoxical contraction, 40 
Paraesthesiae, 36 

Paralysis, distribution of, in infantile 
cerebral palsies, 433 

Erb's, 210 

facial, 216 

infantile spinal, 249 

in peripheral nerve disease, 209 

in spinal palsies, 249 

of ocular muscles in disseminated scle- 
rosis, 307 
Paramyoclonus multiplex, 141 
Paranoia, 534 
Paraplegia, spastic cerebral, 430 

spastic spinal, 347 
Paretic dementia, 538 
Parietal lobe, tumor of, 476 
Patellar reflex, 40 
Pavor nocturnus, 188 
Periodic insanity, 532 
Peripheral nerves, diseases of, 209 

palsies, 209, 225 
Peroneal paralysis, 225 

form of progressive muscular atrophy, 

359 
Petit mal, 68 
Pleuroplegia, 516 

congenital, 517 
Polio-encephalitis superior, 417 
Polio-encephalomyelitis, 418 
Poliomyelitis anterior acuta, 249 
Polyneuritis, 238. See Multiple neuri- 
tis. 
Pons, tumor of, 478 
Porencephaly, 502 

acquired, 505 

congenital, 503 
Post-hemiplegic chorea, 117, 442 
Pott's paralysis, 3, 315 

diagnosis of, 318 

symptoms of, 317 
Primary dystrophies, types of, 369 

muscular dystrophies, 368 

myopathies, 368 
Progressive amyotrophy, 355 
Progressive muscular atrophies, 352 

pathology of, 356 

hereditary form, 357 

muscular atrophy, abortive and hered- 
itary forms of, 359 
Progressive neural muscular atrophy, 

359 
neurotic muscular atrophy, 359 
pathology of, 366 
Pseudo-hypertrophic muscular paraly- 
sis, 371 
Pseudo-bulbar palsy, 420 
Ptosis, 11, 241 
Pupil inequality, 12 



Raynaud's disease, 203 
Reaction of degeneration, 44 
Reflex of cornea, test for, 10 
Reflexes, examination of, 40 

variations of, 41 
Rest, efficacy of, in chorea, 124 
Rheumatic nodules in chorea, 116 
Risus sardonicus, 145 

Sarcoma of brain, 483 
Scaphocephalus, 6 
Sclerosis, amyotrophic lateral, 420 
Sensation, disturbances of, in migraine, 
176 

tests for, 37 

varieties of, 37 
Sensory disturbances, determination of, 

36, 37 
Sensory nerves, distribution of, 38, 39 
Skull, abnormalities of, 6 

measurements of, 4 
Sleep, disorders of, 186 

normal amount of, 187 
Somnambulism, 191 
Spasms, 135 et seq. 
Spastic hemiplegia, 430 

diplegia, 396, 430 

paraplegia, 430 

spinal paralysis, 347 
Speech, difficulties of, in maladie des tics 

convulsifs, 138 
Speech, disturbance of, in hereditary 
chorea, 131 

disturbances of, in chorea, 114 
Spina bifida, 387 
Spinal cord, gliosis of, 329 

hereditary or family diseases of, 331 

injuries of, 288 

localization in. See Appendix. 

malformations and conditions due to 
defective development of, 386 

syphilis of, 293 

tumors of, 321 
Spotted fever, 404 
Stellwag's symptom in exophthalmic 

goitre, 194 
Subacute anterior poliomyelitis, 270 
Subjective sensations, 36 
Sydenham's disease, 108 
Syphilis of spinal cord, morbid anatomy 
of, 299 

symptoms of, 293 

treatment of, 302 
Syringomyelia, 329 
Syringo-myelocele, 388 

Tabes dorsalis spasmodique, 350 
Tachycardia, 199 

Teeth, significance of condition of, 13 
Temperature-sense, 37 
Temporo-sphenoidal lobe, tumor of, 476 



INDEX. 



571 



Tendon reflexes, 40 
Tetanilla, 156. See Tetany. 
Tetanoid chorea, 164 
Tetanus, 143 

bacillus of, 150 

differential diagnosis of, 152 

etiology of, 141 

pathology of, 149 

prognosis of, 153 

symptoms of, 145 

treatment of, 153 

varieties of, 147 
Tetanus neonatorum, 143, 147 
Tetany, 156 

etiology of, 159 

latent period of, 160 

pathology of, 161 

symptoms of, 157 

treatment of, 163 
Thomsen's disease, 139 
Thrombosis, of intra-cranial sinuses, 

497 

of lateral sinus, differential diagnosis 
from brain abscess, 499 
Thyroid enlargement at puberty, 199 
Thyroid gland, extirpation of, in relation 
to tetany, 162 

feeding in myxcedema, 201, 544 
Tic convulsif, 137, 225 
Tics, 134 
Topoalgia, 36 
Torticollis, spasmodic, 225 
Toxic headaches, 174 

injuries of spinal cord, 288 
Tremor, fibrillary, 354 

of multiple sclerosis, 304 
Trigeminal neuralgia, rarity of, 182 
Trismus, 143 

Trophic disturbances in peripheral neu- 
ritis, 209 
Trophic symptoms, occurrence of, 45 
Tropho-neuroses, 192 
Trousseau's symptom, 160 
Tubercular meningitis, 397 

morbid anatomy, 400 



Tubercular meningitis, onset, 397 

symptoms, 397 
Tumor of cortex, 474 

of basal ganglia, 477 

of cerebellum, 479 

of corpora quadrigemina, 477 

of crus cerebri, 477 

frontal lobe, 475 

of medulla and pons, 478 

of motor area, 475 

of occipital lobe, 476 

of parietal lobe, 476 

of temporo-sphenoidal lobe, 476 

of third frontal convolution, 475 
Tumors of the brain and meninges, 470 

differential diagnosis, 480 

pathology of, 481 

treatment of, 484 
Tumors of spinal cord and meninges, 
320 

diagnosis of, 325 

prognosis, 327 

symptoms ®f, 320 

treatment of, 327 



Uraemia, headaches due to, 173 
Urine, changes in, in chorea, 116 



Vasomotor neuroses, 192 

Ventricle, fourth, stimulation of floor of, 

79 
Vertigo in migraine, 177 
Vision, disturbances of, in hysteria, 

100 
test for, in infants, 8 
Visual field, 9 

Weight-sense, 37 

Weir-Mitchell plan of treatment, 105 

Wrist-drop, 21, 241 

Wry-neck, 225 

congenital, 225 

symptomatic, 225 



H 11 88 







V* 




^ 











-ov*" : 




* s ^, 



A <, *<T.«< .6* V '^•». < \/V <, "'TV. 4 -0 V 'o, 







3* 



V ^ 




• A><^ - 









% 




^ .% 







r .* 




?■*+ 



\/ 




.«L m A^+ % jy 








V* . *V*"^\g* v 'V-ra\<v* %*^^ aC ,* v 



v«,v 




^ 



o_ * 






1 






^^- 










»°*«, 



♦ O* 4T »°'** ^ 








^°* 




» ^ 










HECKMAN 

BINDERY INC. 



SEPT 88 



N. MANCHESTER, 
^^ INDIANA 46962 







a* <^ V@IS»" «J»"~^ 







$*r 



g°V-- 




r oV 



^o 1 



